Author Affiliations

1. CIRO, Center of Expertise for Chronic Organ Failure, Horn, The Netherlands
2. Department of Respiratory Medicine, Maastricht University Medical Center, Maastricht, The Netherlands
3. Centre of Expertise for Palliative Care, Maastricht University Medical Center, Maastricht, The Netherlands
4. COPD Center, Sahlgrenska University Hospital and Institute of Medicine, Gothenburg University, Gothenburg, Sweden

Address correspondence to:

Dr. Sarah Houben-Wilke
Hornerheide 1
6085 NM Horn
The Netherlands
Email: sarahwilke@ciro-horn.nl
Phone: +31 (0)475 587 602

Abstract

Beyond respiratory impairment, patients with chronic obstructive pulmonary disease (COPD) often suffer from comorbidities which are associated with worse health status, higher health care costs and worse prognosis. Reported prevalences of comorbidities largely differ between studies which might be explained by different assessment methods (objective assessment, self-reported assessment, or assessment by medical records), heterogeneous study populations, inappropriate control groups, incomparable methodologies, etc. This narrative review demonstrates and further evaluates the variability in prevalence of several comorbidities in patients with COPD and control individuals and discusses several shortcomings and pitfalls which need to be considered when interpreting comorbidity data. Like in other chronic organ diseases, the accurate diagnosis and integrated management of comorbidities is a key for outcome in COPD. This review highlights that there is a need to move from the starting point of an established index disease towards the concept of the development of multimorbidity in the elderly including COPD as an important and highly prevalent pulmonary component.

Citation

Citation: Houben-Wilke S, Triest FJJ, Franssen FME, Janssen DJA, Wouters EFM, Vanfleteren LEGW. Revealing methodological challenges in chronic obstructive pulmonary disease studies assessing comorbidities: a narrative review. J COPD F. 2019; 6(2): 166-177. doi: http://doi.org/10.15326/jcopdf.6.2.2018.0145

Keywords

COPD, chronic obstructive pulmonary disease, comorbidity, multimorbidity, methodology, bias, control group

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Click to read Revealing Methodological Challenges in Chronic Obstructive Pulmonary Disease Studies Assessing Comorbidities: A Narrative Review

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Author Affiliations

1. Division of Pulmonary, Critical Care, and Sleep Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
2. Institute for Health Care Delivery Science at Department of Population Health Science and Policy, Icahn School of Medicine at Mount Sinai, New York, New York

Address correspondence to:

Sidney Braman, MD
Division of Pulmonary, Critical Care & Sleep Medicine
Icahan School of Medicine at Mount Sinai
5th Floor, Rm 5-20
Annenberg Bldg
1468 Madison Avenue
New York, NY 10029
Email: sidney.braman@mssm.edu

Abstract

We evaluated whether visiting a primary care provider (PCP) or medical subspecialist within 10 days of discharge reduces 30-day readmissions following hospitalization for acute exacerbation of chronic obstructive pulmonary disease (AECOPD). Data were retrospectively collected from electronic health records for AECOPD-related hospitalizations at an urban, academic medical center for patients 40 years of age or older between June 2011 and June 2016. Primary outcome was probability of all-cause 30-day readmission. Follow-up was defined as visiting a PCP or any medical subspecialist within 10 days of discharge. Generalized linear mixed models were used to examine the association between hospital readmissions and a visit to a PCP or medical subspecialist. Of the 2653 hospital discharges, 17.6% (n=468) had a 30-day readmission. Follow-up did not affect 30-day readmission risk (adjusted odds ratio 1.14; 95% confidence interval 0.89, 1.47). Prompt follow-up is not associated with a reduced risk of 30-day readmission following AECOPD, highlighting the need for a comprehensive approach to chronic obstructive pulmonary disease (COPD).

Citation

Citation: Budde J, Agarwal P, Mazumdar M, Braman SS. Follow-up soon after discharge may not reduce COPD readmissions. J COPD F. 2019; 6(2): 129-131. doi: http://doi.org/10.15326/jcopdf.6.2.2018.0149

Keywords

COPD, chronic obstructive pulmonary disease, hospital readmissions, acute exacerbation of COPD, AECOPD, physician follow-up, health care delivery

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Click to read Follow-up Soon After Discharge May Not Reduce COPD Readmissions

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Author Affiliations

1. Denver, Colorado

Address correspondence to:

Ron Balkissoon, MD, MSc, DIH, FRCPC
balkissoonr@njhealth.org

Abstract

This article does not contain an abstract.

Citation

Citation: Balkissoon R. Journal club: endobronchial valve bronchoscopic lung volume reduction. J COPD F. 2019; 6(1): 118-125. doi: http://doi.org/10.15326/jcopdf.6.1.2019.0126

Keywords

COPD, emphysema, chronic obstructive pulmonary disease, lung volume reduction, endobronchial valve

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Click to read Journal Club: Endobronchial Valve Bronchoscopic Lung Volume Reduction

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Author Affiliations

1. St Francis Hospital and University Medical Research, LLC

Address correspondence to:

Nicholas Gross, MD, PhD
Grossnicholas1@gmail.com

Abstract

This article does not contain an abstract.

Citation

Citation: Gross N. COPD pipeline XXXX. Chronic Obstr Pulm Dis. J COPD F. 2019; 6(1): 115-117. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0165

Keywords

Gala Airway, MyCOPD, dual orexin receptor antagonist, triple therapy

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Click to read The COPD Pipeline XXXX

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Author Affiliations

1. Education Institute, Cleveland Clinic, Cleveland, Ohio

Address correspondence to:

James K. Stoller, MD, MS
Cleveland Clinic
9500 Euclid Ave
Cleveland, Ohio 44195
Phone: (216) 444-1960
Email: stollej@ccf.org

Abstract

This article does not contain an abstract.

Citation

Citation: Stoller JK. Editorial. Progress in alpha-1 antitrypsin deficiency: collaboration as the foundation of new knowledge. J COPD F. 2019; 6(1): 1-5. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0164

Keywords

chronic obstructive pulmonary disease, alpha-1 antitrypsin deficiency

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Click to read Progress in Alpha-1 Antitrypsin Deficiency: Collaboration as the Foundation of New Knowledge

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Author Affiliations

1. Department of Internal Medicine, University of Michigan, Ann Arbor
2. Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor
3. Division of Pulmonary Medicine, National Jewish Health, Denver, Colorado
4. Division of Pulmonary and Critical Care Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania
5. Division of Pulmonary, Critical Care and Sleep Medicine, University of California, San Diego
6. Division of Pulmonary, Allergy and Critical Care Medicine, University of Alabama, Birmingham
7. School of Public Health, University of Michigan, Ann Arbor
8. School of Public Health, University of Colorado, Aurora
9. Channing Division of Network Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
10. Veterans’ Administration Ann Arbor Healthcare System, Ann Arbor, Michigan

Address correspondence to:

MeiLan Han, MD, MS
Division of Pulmonary and Critical Care Medicine
3916 Taubman Center
1500 E. Medical Center Drive
University of Michigan Medical Center
Ann Arbor, MI 48109-0360
Phone: 734-763-2540
Email: Mrking@med.umich.edu

Abstract

Background: The Global initiative for chronic Obstructive Lung Disease (GOLD) ABCD groupings were recently modified. The GOLD 2011 guidelines defined increased risk as forced expiratory volume in 1 second (FEV₁) < 50% predicted or ≥ 2 outpatient or ≥ 1 hospitalized exacerbation in the prior year, whereas the GOLD 2017 guidelines use only exacerbation history. We compared mortality and exacerbation rates in the Genetic Epidemiology of COPD Study cohort (COPDGene^®) by 2011 (exacerbation history/FEV₁ and dyspnea) versus 2017 (exacerbations and dyspnea) classifications.

Methods: Using data from COPDGene^®, we tested associations of ABCD groups with all-cause mortality (Cox models, adjusted for age, sex, race and comorbidities) and longitudinal exacerbations (zero-inflated Poisson models).

Results: In 4469 individuals (mean age 63.1 years, 44% female), individual distributions in 2011 versus 2017 systems were: A, 32.0% versus 37.0%; B, 17.6% versus 36.3%; C, 9.4% versus 4.4%; D, 41.0% versus 22.3%; (observed agreement 76% [expected 27.8%], Kappa 0.67, p<0.001). Individuals in group D-2011 had 1.1 ± 1.6 exacerbations/year (mean ± standard deviation [SD]) versus 1.4 ± 1.8 for D-2017 (median follow-up 3.7 years). Using group A as reference, for both systems, mortality (median follow-up 6.8 years) was highest in group D (D-2011, [hazard ratio] HR 5.2 [95% confidence interval (CI) 4.2, 6.4]; D-2017, HR 5.5 [4.5, 6.8]), lowest for group C (HR 1.9 [1.4, 2.6] versus HR 1.9 [1.3, 2.8]) and intermediate for group B (HR 2.6 [2.0, 3.4] versus HR 3.4 [2.8, 4.1]). GOLD 2011 had better mortality discrimination (area under the curve [AUC] 0.68) than GOLD 2017 (AUC 0.66, p<0.001 for comparison) but similar exacerbation rate prediction.

Conclusions: Relative to the GOLD 2011 consensus statement, discriminate predictive power of the 2017 ABCD classification is similar for exacerbations but lower for survival.

Citation

Citation: Criner RN, Labaki WW, Regan EA, et al; for the COPDGene Investigators. Mortality and exacerbations by Global Initiative for Chronic Obstructive Lung Disease Groups ABCD: 2011 versus 2017 in the COPDGene® Cohort. J COPD F. 2019; 6(1): 64-73. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0130

Keywords

COPD, chronic obstructive pulmonary disease, Global initiative for chronic Obstructive Lung Dis, exacerbation mortality

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Click to read Mortality and Exacerbations by Global Initiative for Chronic Obstructive Lung Disease Groups ABCD: 2011 Versus 2017 in the COPDGene® Cohort

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Author Affiliations

1. Division of Pulmonary and Critical Care, School of Medicine, Johns Hopkins University, Baltimore, Maryland
2. Department of International Health, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland
3. Department of Physiology, College of Health Sciences, Makerere University, Kampala, Uganda
4. UCL Respiratory, University College London, United Kingdom
5. Department of Medicine and Lung Institute, College of Health Sciences, Makerere University, Kampala, Uganda

Address correspondence to:

Nicole Robertson
Email: Nicolemrobertson01@gmail.com
Phone:(859) 468-9147

Abstract

Introduction: Almost 90% of chronic obstructive pulmonary disease (COPD) deaths occur in low- and middle-income countries (LMICs), where there are large rural populations and access to health care for COPD is poor. The purpose of this study was to compare urban-rural provider experiences regarding systemic facilitators and barriers to COPD management and treatment access.

Methods: We conducted a qualitative study using direct observations and in-depth semi-structured interviews with 16 and 10 health care providers in urban Kampala and rural Nakaseke, Uganda, respectively. We analyzed interviews by performing inductive coding using generated topical codes.

Results: In both urban and rural districts, exposure to evidence-based practices for COPD diagnosis and treatment was limited. The biomedical definition of COPD is not well distinguished in rural communities and was commonly confused with asthma and other respiratory diseases. Urban and rural participants alike described low availability of medications, limited access to diagnostic tools, poor awareness of the disease, and lack of financial means for medical care as common barriers to seeking and receiving care for COPD. While there was greater access to COPD treatment in urban areas, rural populations faced more pronounced barriers in access to diagnostic equipment, following standard treatment guidelines, and training medical personnel in non-communicable disease (NCD) management and treatment.

Conclusion: Our results suggest that health system challenges for the treatment of COPD may disproportionately affect rural areas in Uganda. Implementation of diagnostic and treatment guidelines and training health professionals in COPD, with a special emphasis on rural communities, will assist in addressing these barriers.

Citation

Citation: Robertson NM, Nagourney EM, Pollard SL, et al. Urban-rural disparities in chronic obstructive pulmonary disease management and access in Uganda. J COPD F. 2019; 6(1): 17-28. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0143

Keywords

COPD, chronic obstructive pulmonary disease, Urban-rural health disparities, treatment access, low and middle-income countries, Uganda

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Click to read Urban-Rural Disparities in Chronic Obstructive Pulmonary Disease Management and Access in Uganda

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Author Affiliations

1. Research Department, COPD Foundation, Inc., Washington, D.C.
2. Patient & Health Impact, Pfizer, Inc., Collegeville, Pennsylvania
3. College of Public Health, University of Kentucky Lexington, and Consultant, COPD Foundation, Inc.
4. Medical Affairs, Pfizer, Inc., Columbia, Missouri
5. Medical Affairs, Pfizer, Inc., Lexington, Kentucky

Address correspondence to:

Cara B. Pasquale, MPH
Phone: 866-731-2673, ext. 312
Email: cpasquale@copdfoundation.org

Abstract

Community acquired pneumonia (CAP) carries high morbidity, mortality, and economic burden, which is even higher in adults diagnosed with chronic obstructive pulmonary disease (COPD). While several studies have assessed the clinical burden and mortality risk of CAP and COPD, very few studies focus on CAP burden from a COPD patient perspective. Individuals recently diagnosed with CAP and with pre-existing COPD were recruited through the COPD Foundation. The CAP Burden of Illness Questionnaire (CAP-BIQ), a content validated questionnaire assessing CAP symptomatology, duration of symptoms and CAP impact on work, activities and family, was administered at baseline and at 30-days follow-up.

Of the 490 participants recruited, 481 had data sufficient for analysis. The prevalence of respiratory-related symptoms was very high (>90%) at the time of diagnosis with other generalized symptoms such as fatigue, trouble sleeping, headaches and confusion present in more than 60% of participants. Mean duration of symptoms varied from approximately 2 weeks for headaches and fever to more than a month for fatigue, wheezing, dyspnea, and cough. Employed participants missed an average of 21 days of work and those not employed missed 36 days of usual activities. Over 84% required help from family, friends or care givers.

CAP is a serious and burdensome condition for people with COPD, a condition that can impair activities for weeks, frequently requires care from family or friends, and includes lingering symptoms. The patient-reported impact of CAP reported in this study underscores the need for prevention strategies in this population.

Citation

Citation: Pasquale CB, Vietri J, Choate R, et al. Patient-reported consequences of community-acquired pneumonia in patients with chronic obstructive pulmonary disease. J COPD F. 2019; 6(2): 132-144. doi: http://doi.org/10.15326/jcopdf.6.2.2018.0144

Keywords

COPD, patient reported outcomes, symptom burden, community acquired pneumonia, time to resolution

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Click to read Patient-Reported Consequences of Community-Acquired Pneumonia in Patients with Chronic Obstructive Pulmonary Disease

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Author Affiliations

1. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania
2. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine and Temple School of Pharmacy, Temple University, Philadelphia, Pennsylvania
3. Department of Clinical Sciences, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania

Address correspondence to:

Gerard J. Criner, MD
Department of Thoracic Medicine and Surgery
Lewis Katz School of Medicine at Temple University
745 Parkinson Pavilion
3401 North Broad Street
Philadelphia Pa 19140
Phone: 215 707-8113
Email: gerard.criner@tuhs.temple.edu

Abstract

Introduction: Hospitalization for chronic obstructive pulmonary disease (COPD) exacerbation portends the greatest risk of rehospitalization and mortality. Treatments that prevent hospitalizations could significantly lessen COPD morbidity and mortality.

Methods: We performed a prospective, randomized, double-blind, placebo-controlled study of roflumilast 500 ug daily versus placebo in patients hospitalized for acute COPD exacerbation. Primary outcome was time to all-cause mortality or non-elective rehospitalization at 180 days post-randomization. Secondary outcomes were death or hospitalization from a respiratory cause, quality of life, change in health status, forced expiratory volume in 1 second (FEV₁) and roflumilast tolerance.

Results: A total of 64 patients with moderate to severe COPD (FEV₁, 37.6 ± 16.4% predicted; 61% female, 61.6 ± 7.9 years old) were assigned to roflumilast or placebo. No deaths occurred in the follow-up period. There was no difference in the time to first readmission between the roflumilast and placebo groups (46.1 days versus 47.3 days respectively, p=0.93). There were 29 and 30 readmissions in the roflumilast and placebo groups, respectively (p=0.47). The St George’s Respiratory Questionnaire (SGRQ) decreased 10.8 points and 7.8 points in the roflumilast and placebo groups, respectively and were not different. EuroQuality of Life Five Dimension scale (EQ5D) scores improved, but not significantly in either group. Weight loss and nausea were more common with roflumilast but not different from placebo. Change in glycosylated hemoglobin percentage (HgbA1C%) was not different between groups. Sub-analysis for the impact of chronic bronchitis did not affect outcomes.

Conclusion: In this pilot study conducted in patients hospitalized with an exacerbation of COPD, roflumilast did not affect time to all-cause rehospitalization, quality of life, FEV₁ or any other measured parameter.

Citation

Citation: Criner GJ, Jacobs MR, Zhao H, Marchetti N.Effects ofroflumilast on rehospitalization and mortality in patients hospitalized with a COPD exacerbation. J COPD F. 2019; 6(1): 74-85. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0139

Keywords

exacerbations, roflumilast, COPD hospitalization

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Click to read Effects of Roflumilast on Rehospitalization and Mortality in Patients

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Author Affiliations

1. Pulmonary Research Institute of Southeast Michigan, Farmington Hills
2. Sunovion Pharmaceuticals Inc., Marlborough, Massachusetts

Address correspondence to:

Gary T. Ferguson, MD,
Pulmonary Research Institute of Southeast Michigan
29255 West 10 Mile Road, Suite A
Farmington Hills, MI 48336
Phone: 248-478-6561
E-mail: garytferguson@msn.com

Abstract

Purpose: The purpose of this study was to assess the effect of pre-existing cardiovascular (CV) risk factors on the efficacy and safety of nebulized glycopyrrolate (GLY) in patients with chronic obstructive pulmonary disease (COPD).

Methods: A total of 2379 patients from 3 phase III studies (12-week, placebo-controlled Glycopyrrolate for Obstructive Lung Disease via Electronic Nebulizer [GOLDEN] -3 and -4, and 48-week, active-controlled GOLDEN-5) stratified by high (n=1526) or low (n=853) CV risk were randomized to placebo, GLY 25 mcg or 50 mcg twice daily, or tiotropium (TIO; 18 mcg once daily). Safety, lung function, patient-reported outcomes (PROs), and exacerbations were assessed by CV risk.

Results: Treatment-emergent adverse events (TEAEs) were similar across CV risk subgroups, with serious TEAEs higher in the high CV risk subgroup. In the 12-week studies, discontinuation due to TEAEs with GLY 25 mcg and 50 mcg was similar between CV risk subgroups, and lower than placebo (high risk: 6.2%, 3.6%, 9.0%; low risk: 3.2%, 4.5%, 9.9%, respectively). In the 48-week, open-label study, discontinuation rates were higher with GLY versus TIO (high risk: 10.7%, 3.7%; low risk: 8.7%, 1.2%, respectively). Rates of CV events of special interest were similar across CV risk subgroups. Regardless of CV risk, GLY led to significant improvements in efficacy and PRO assessments at 12 weeks versus placebo, whereas changes were similar between GLY and TIO at 48 weeks, except for PROs in the low risk subgroup. Exacerbation rates were similar across all treatment groups.

Conclusions: Nebulized GLY had an acceptable safety profile and improved lung function and PROs in COPD patients, irrespective of CV risk status.

Citation

Citation: Ferguson GT, Tosiello R, Sanjar S, Goodin T. Efficacy and safety of nebulized glycopyrrolate/eflow® closed system in patients with moderate-to-very-severe chronic obstructive pulmonary disease with pre-existing cardiovascular risk factors. J COPD F. 2019; 6(1): 86-99. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0146

Keywords

COPD, chronic obstructive pulmonary disease, glycopyrrolate, eFlow, nebulized long-acting muscarinic antagonist, LAMA, cardiovascular

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Click to read Efficacy and Safety of Nebulized Glycopyrrolate/eFlow® Closed System in Patients with Moderate-to-Very-Severe Chronic Obstructive Pulmonary Disease with Pre-Existing Cardiovascular Risk Factors

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Author Affiliations

1. RTI Health Solutions, Research Triangle Park, North Carolina
2. Pulmonx Corporation, Redwood City, California
3. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania
4. Division of Pulmonary and Critical Care Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts

Address correspondence to:

Carol Mansfield
RTI Health Solutions
200 Park Offices Drive
Research Triangle Park, NC, 27709
Email:carolm@rti.org
Phone: 919.541.8053

Abstract

Background: Patients with severe emphysema have limited treatment options. Little is known about patients’ willingness to accept risks for new treatments that offer meaningful benefits.

Methods: We determined treatment preferences of patients with severe emphysema using a web-based discrete-choice experiment survey. Respondents answered 9 questions that offered choices between 2 hypothetical interventional treatments or continuing current medical management. Variations in 5 attributes defined the 2 interventional treatments: improvement in ability to breathe and carry out day-to-day activities, frequency of hospitalized exacerbations, treatment type, risk of pneumothorax within 30 days of procedure, and risk of death within 3 months. Respondents were recruited through the COPD Foundation’s COPD Patient-Powered Research Network and had a self-reported emphysema diagnosis and 2+ score on the modified Medical Research Council Dyspnea Scale. The relative importance of the attributes and the percentage of respondents who would select different treatment options was modeled using random-parameters logit.

Results: Among 294 respondents, 51% always chose an interventional treatment option, while 19% always selected continued medical management. The most important change on average was moving from continued medical management (with no improvement in breathlessness) to an interventional treatment with improvement in breathlessness. The model predicted 71% of respondents would select a treatment option similar to removable endobronchial valve implants, 6% would select lung volume reduction surgery (LVRS), and 23% continued medical management.

Conclusion: Patients with severe emphysema perceive that a procedure with risks and benefits similar to the Zephyr^® endobronchial valve implants is desirable over continued medical management or LVRS.

Citation

Citation: Mansfield C, Sutphin J, Shriner K, Criner GJ, Celli BR. Patient preferences for endobronchial valve treatment of severe emphysema. J COPD F. 2019; 6(1): 51-63. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0147

Keywords

COPD, emphysema, chronic obstructive pulmonary disease, lung volume reduction surgery, endobronchial valves, patient preferences, discrete choice experiment

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Click to read Patient Preferences for Endobronchial Valve Treatment of Severe Emphysema

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Author Affiliations

1. Department of Internal Medicine, University of Michigan, Ann Arbor
2. Department of Clinical Sciences, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania
3. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania

Address correspondence to:

Rachel N. Criner, MD
Department of Internal Medicine
University of Michigan Health Systems
UH Internal Medicine – Core 3116 TC
1500 E. Medical Center Drive
Ann Arbor, MI 48109
rcriner@med.umich.edu
734-936-4385

Abstract

Abstract

Background: Long-term effects of lung volume reduction surgery (LVRS) on respiratory muscle strength and effects of age, sex, and emphysema pattern on these changes are unknown. Therefore, we aimed to determine the long-term effect of LVRS on respiratory muscle strength changes in severe emphysema.

Methods: The National Emphysema Treatment Trial was a prospective controlled multicentered trial, comparing LVRS to optimal medical treatment on survival and maximal exercise capacity. We examined percentage change in maximum inspiratory pressure (MIP) from baseline to 36 months follow-up to determine impact of LVRS as well as age, sex, emphysema pattern and exercise capacity on changes in MIP compared to medical treatment.

Results: LVRS individuals had significantly greater increases in MIP from baseline compared to medical individuals at all follow-ups (LVRS 19.8 ± 42.3%, medical 3.2 ± 29.3%, p<0.0001, 12 months). The LVRS group had significant decreases in total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC) and RV/TLC compared to the medical arm at all follow-up periods. Males and individuals 65-70 years of age had significantly greater increases in MIP following LVRS compared to the medical arm at all follow-ups; this same relationship was seen at up to 24 months for low exercise capacity, upper lobe predominant emphysema.

Conclusions: LVRS significantly increases inspiratory muscle strength up to 3 years post-operatively, with male sex, age 65-70 years and low exercise capacity, upper lobe predominant emphysema especially associated with increased MIP. Inspiratory muscle strength increases were associated with decreases in non-invasive markers of dynamic hyperinflation, suggesting that LVRS allows inspiratory muscles to return to their optimal length-tension relationship.

Citation

Citation: Criner RN, Yu D, Jacobs MR, Criner GJ. Effect of lung volume reduction surgery on respiratory muscle strength in advanced emphysema. J COPD F. 2019; 6(1): 40-50. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0188

Keywords

emphysema, exercise capacity, lung volume reduction surgery, dynamic hyperinflation, respiratory muscle strength, mean inspiratory pressure, sex, age

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Click to read Effect of Lung Volume Reduction Surgery on Respiratory Muscle Strength in Advanced Emphysema

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Author Affiliations

1. Department of Preventive Medicine and Environmental Health, University of Kentucky College of Public Health, Lexington
2. Division of Medical, Behavioral, and Community Health, National Jewish Health; Department of Community and Behavioral Health, University of Colorado Denver School of Public Health, Denver
3. Division of Pulmonary Sciences and Critical Care Medicine, National Jewish Health, University of Colorado Denver School of Medicine, Denver

Address correspondence to:

Radmila Choate, MPH
Department of Preventive Medicine and Environmental Health
University of Kentucky College of Public Health
111 Washington Avenue
Lexington, KY 40536
Phone: 859-218-2237
E-mail: radmila.choate@uky.edu

Abstract

Background: The aim of this study was to examine differences in demographic, health, and behavioral characteristics in individuals with ZZ and SZ genotypes of alpha-1 antitrypsin deficiency (AATD) within AlphaNet’s Disease Management and Prevention Program (ADMAPP).

Methods: Self-reported data from 3535 patients with AATD, including 3031 (85.7%) patients with ZZ, ZNull, and NullNull genotypes (referred to here as ZZ), and 504 (14.3%) with the SZ genotype were analyzed using t-tests, ANOVAs, and Chi-squared tests.

Results: The average age of the cohort was 56.3±10.6 years. The majority of respondents were male (51.2%), white (98.2%) and married (65.2%). SZs reported having more frequent exacerbations (p<0.001) and hospitalizations (p=0.012) than ZZs. A higher proportion of SZs than ZZs had been diagnosed with high blood pressure, diabetes, congestive heart failure, and other comorbid conditions. SZs were more likely than ZZs to report “poor” health (p=0.005). Over a third (38.4%) of SZs do not exercise compared to 27.1% of ZZs (p<0.001). A greater proportion of SZs compared to ZZs view themselves as being overweight (p<0.001) or “out of shape” (p=0.001). A higher proportion of SZs than ZZs reported any history of smoking and current smoking (p<0.001).

Conclusions: In patients with AATD and lung disease participating in a disease management program, a higher proportion of SZs than ZZs report exacerbations, comorbidities, and overall poor health, as well as unhealthy behaviors such as lack of exercise and current smoking. Future work should consider the extent to which genotype-specific health promotion interventions would be useful.

Citation

Citation: Choate R, Mannino DM, Holm KE, Sandhaus RA. Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: findings from AlphaNet’s disease management program. J COPD F. 2019; 6(1): 29-39. doi: http://doi.org/10.15326/jcopdf.6.1.2018.0134

Keywords

alpha-1 antitrypsin deficiency, genotype, AlphaNet Disease Management and Prevention Program, ADMAPP, ZZ, SZ

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Click to read Comparing Patients with ZZ Versus SZ Alpha-1 Antitrypsin Deficiency: Findings from AlphaNet’s Disease Management Program

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Author Affiliations

1. Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville

Address correspondence to:

Mark L. Brantly, MD
Division of Pulmonary, Critical Care, and Sleep Medicine
College of Medicine
University of Florida
JHMHC PO Box 100225
Gainesville, FL 32610
Email: mbrantly@ufl.edu
Phone: (352)273-8737

Abstract

Alpha-1 antitrypsin deficiency (AATD) is a largely monogenetic disorder associated with a high risk for the development of chronic obstructive pulmonary disease (COPD) and cirrhosis. Intravenous alpha-1 antitrypsin (AAT) therapy has been available for the treatment of individuals with AATD and COPD since the late 1980s. Initial Food and Drug Administration (FDA) approval was granted based on biochemical efficacy. Following its approval, the FDA, scientific community and third-party payers encouraged manufacturers of AAT therapy to determine its clinical efficacy. This task has proved challenging because AATD is a rare, orphan disorder comprised of individuals who are geographically dispersed and infrequently identified. In addition, robust clinical trial outcomes have been lacking until recently. This review provides an update on the evidence for the clinical efficacy of intravenous AAT therapy for patients with AATD-related emphysema.

Citation

Citation: Brantly ML, Lascano JE, Shahmohammadi A. Intravenous alpha-1 antitrypsin therapy for alpha-1 antitrypsin deficiency: the current state of the evidence.

Keywords

COPD, emphysema, alpha-1 antitrypsin deficiency, AAT therapy, clinical efficacy, disease modification

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Click to read Intravenous Alpha-1 Antitrypsin Therapy for Alpha-1 Antitrypsin Deficiency: The Current State of the Evidence

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Author Affiliations

1. Pharmaceutical Health Services Research Department, School of Pharmacy, University of Maryland, Baltimore
2. OptumLabs, Cambridge, Massachusetts
3. Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, Colorado
4. School of Medicine, University of Maryland, Baltimore
5. Division of Medical, Behavioral, and Community Health, National Jewish Health, Denver, Colorado

Address correspondence to:

C. Daniel Mullins, PhD
Pharmaceutical Health Services Research Department
University of Maryland School of Pharmacy
220 N Arch St, 12^th Floor
Baltimore, MD 21201-1531 Email: Daniel.Mullins@rx.umaryland.edu

Abstract

Background: This study is the first to utilize a large claims database to estimate medical costs of patients with alpha-1 antitrypsin deficiency (AATD) in the United States.

Methods: Adult AATD patients were identified from the OptumLabs™ Data Warehouse. Insurer and patient out-of-pocket costs were categorized into the following cost buckets, stratified by augmentation therapy use: physician visits (PV), emergency department visits (ED), inpatient stays (IP), augmentation therapy (AUG), other prescription drug costs (RX), and other costs (OTH). Costs were weighted and adjusted to 2017 U.S. dollars using the medical care component of the consumer price index.

Results: The study cohort consisted of9117 AATD patients followed for 53,872 person years observed between 1993 and 2015. The annual costs among AATD patients totaled $127,537 among augmentation therapy users and $15,874 among non-users. The major drivers of annual costs to the insurer among the 7975 patients not on augmentation therapy were: PV: $5352 (37.7%) and IP: $4506 (31.8%). Among the 1142 augmentation users, major annual cost drivers to the insurer were PV: $15,064 (12.3%) and AUG: $82,002 (66.7%). Annual patient out-of-pocket costs were $4601 (AUG: $2084 [45.3%]; RX: $940 [20.4%]) and $1689 (PV: $727 [43.0%]; RX: $589 [34.9%]) among augmentation therapy users and non-users, respectively. Averaged across the entire cohort, the average annual costs per AATD patient were $22,975, paid by insurers ($21,100) and patients ($1875).

Conclusions: Annual medical costs among patients with AATD are $127,537 and $15,874 among augmentation therapy users and non-users, respectively, with 75.3% of the cost difference attributable to AUG.

Citation

Citation: Sieluk J, Levy J, Sandhaus RA, Silverman H, Holm KE, Mullins CD. Costs of medical care among augmentation therapy users and non-users with alpha-1 antitrypsin deficiency in the United States. J COPD F. 2019; 6(1): 6-16. doi: http://doi.org/10.15326/jcopdf.6.1.2017.0187

Keywords

chronic obstructive pulmonary disease, alpha-1 antitrypsin deficiency, pharmacoeconomics, medical costs, augmentation therapy

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Click to read Costs of Medical Care Among Augmentation Therapy Users and Non-Users with Alpha-1 Antitrypsin Deficiency in the United States

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Author Affiliations

1. COPD Foundation, Washington, DC

Address correspondence to:

Elisha Malanga
COPD Foundation
Email: emalanga@copdfoundation.org
Phone: 866-731-2673 x309

Abstract

This article does not contain an abstract.

Citation

Citation: Malanga E, Malanga V, Latham S. Websites, online communities and digital channels as a medium to effectively educate, engage and empower patients. J COPD F. 2018; 5(4): 334-337. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0158

Keywords

online communities, websites, digital channels, patient education, patient engagement

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Click to read Websites, Online Communities and Digital Channels as a Medium to Effectively Educate, Engage and Empower Patients

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Author Affiliations

1. Denver, Colorado

Address correspondence to:

Ron Balkissoon, MD, MSc, DIH, FRCPC
balkissoonr@njhealth.org

Abstract

This article does not contain an abstract.

Citation

Citation: Balkissoon R. Journal club - Asthma /chronic obstructive pulmonary disease overlap: fact or fiction? J COPD F. 2018; 5(4): 341-350. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0162

Keywords

COPD, chronic obstructive pulmonary disease, asthma

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Click to read Journal Club - Asthma/Chronic Obstructive Pulmonary Disease Overlap: Fact or Fiction?

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Author Affiliations

1. St Francis Hospital and University Medical Research, LLC

Address correspondence to:

Nicholas Gross, MD, PhD
Grossnicholas1@gmail.com

Abstract

This article does not contain an abstract.

Citation

Citation: Gross N. COPD pipeline XXXIX. J COPD F. 2018; 5(4): 338-340. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0155

Keywords

long-acting muscarinic antagonist, long-acting beta2-agonist, umeclidinium, vilanterol, phosphodiesterase, non-typeable Haemophilus influenza, Moraxella catarrhalis

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Click to read The COPD Pipeline XXXIX

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Author Affiliations

1. MedStar Georgetown University Hospital; Georgetown University Medical Center, Washington, DC
2. Mayo Clinic, Rochester, Minnesota

Address correspondence to:

Anne E. O'Donnell, MD
Phone: 202-444-8830
Email: ODONNELA@gunet.georgetown.edu

Abstract

This article does not contain an abstract.

Citation

Citation: O’Donnell AE, Aksamit TR. Abstracts: 3rd World Bronchiectasis Conference. J COPD F. 2018; 5(4): 302-323. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0132

Keywords

bronchiectasis, nontuberculous mycobacterial lung infections, NTM, 3rd World Bronchiectasis Conference

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Click to read 3rd World Bronchiectasis Conference

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Author Affiliations

1. COPD Foundation, Washington, DC
2. University of Kentucky, College of Public Health, Lexington
3. GlaxoSmithKline, Philadelphia, Pennsylvania

^* contributed equally to this report

Address correspondence to:

Jamie Sullivan, MPH
COPD Foundation
1140 3^rd St. NE
2^nd Floor
Washington, DC 20002
Email: jsullivan@copdfoundation.org
Phone: 866-731-2678 ext. 455

Abstract

Background: Chronic obstructive pulmonary disease (COPD) remains a leading cause of disability and death in the United States. The “COPD in the United States” project gathered data about the impact of COPD to highlight variability across states and provide a single point of access to data for state decision makers, the public health community and advocates. This report provides a summary of COPD-related morbidity and mortality in the United States and individual states during 2014-2015 (some metrics contain data from other years).

Methods: We used data from multiple sources ( the Behavioral Risk Factor Surveillance System [BRFSS], the Centers for Disease Control and Prevention (CDC) Wide-ranging ONline Data for Epidemiologic Research online database, the CDC’s chronic disease indicators data, Centers from Medicare and Medicaid Services Chronic Conditions Data Warehouse, Medical Expenditures Panel Survey and the American Association of Cardiovascular and Pulmonary Rehabilitation Pulmonary Rehabilitation Directory) to estimate 10 national and state-specific COPD metrics (prevalence, hospitalizations and emergency department visits, 30-day hospital readmissions, mortality, vaccinations, smoking prevalence, per capita medical cost, and the number of COPD patients per a pulmonary rehabilitation program) and to calculate average score across the 10 metrics. Additionally, we used BRFSS data to calculate the prevalence of common comorbid diseases among people who also report having a diagnosis of COPD.

Results: During 2014-2015, 5.9% of adults (more than 15.9 million) reported having been told by their health care professional that they had COPD. The age-adjusted prevalence ranged from 3.7% for Puerto Rico and Hawaii, to 12% for West Virginia. The average score, 1 being best and 5 being worst, of the overall COPD burden based on the 10 key metrics ranged from 1.5 for Puerto Rico and Utah to 4.6 for West Virginia.

Conclusion: The level of COPD morbidity and mortality is severe throughout the United States. There is considerable variability in COPD metrics by state. These differences may be useful in identifying and addressing policy gaps in the public health approach to COPD and in implementing the COPD National Action Plan.

Citation

Citation: Sullivan J, Pravosud V, Mannino DM, Siegel K, Choate R, Sullivan T. National and state estimates of COPD morbidity and mortality — United States, 2014-2015. J COPD F. 2018; 5(4): 324-333. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0157

Keywords

COPD, chronic obstructive pulmonary disease, mortality, comorbidities, Behavioral Risk Factor Surveillance System, prevalence, state data, morbidity

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Click to read National and State Estimates of COPD Morbidity and Mortality — United States, 2014-2015

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Author Affiliations

1. Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, Department of Medicine, University of Miami, Miller School of Medicine, Miami, Florida

Address correspondence to:

Adam Wanner, MD
Phone: (305) 243-3045
Email: awanner@alpha1.org

Abstract

This article does not contain an abstract.

Citation

Citation: Wanner A. Editorial. Why publish scientific meeting proceedings? J COPD F. 2018; 5(4): 231-232. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0159

Keywords

alpha-1 antitrypsin deficiency, scientific proceedings, new therapies

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Author Affiliations

Kenneth R. Chapman
Asthma & Airway Centre
University Health Network
University of Toronto
Room 7-451 East Wing, 399 Bathurst Street
Toronto, Ontario M5T 2S8
Phone: 416-603-5499
Email: ken.chapman.airways@gmail.com

Address correspondence to:

1. University of Toronto and Asthma & Airway Centre, University Health Network, Toronto, Ontario

Abstract

This article does not contain an abstract.

Citation

Citation: Chapman KR. Editorial. Increasing awareness of COPD: two steps forward, one step back. J COPD F. 2018; 5(4): 228-230. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0154

Keywords

COPD, chronic obstructive pulmonary disease, awareness, underdiagnosis, gender bias, racial bias

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Click to read Increasing Awareness of COPD: Two Steps Forward, One Step Back

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Author Affiliations

1. Respiratory Division, Department of Medicine, Faculty of Medicine and Health Sciences, University of Sherbrooke, Quebec Canada
2. Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Quebec, Canada

Address correspondence to:

André M. Cantin, MD
Pulmonary Research Unit
Faculty of Medicine, University of Sherbrooke
3001, 12^ième Avenue Nord, Fleurimont, QC
Canada J1H 5N4
Phone number: 819-346-1110 ext. 14893
Email: Andre.Cantin@USherbrooke.ca

Abstract

Antioxidants represent an attractive therapeutic avenue for individuals with chronic obstructive pulmonary disease (COPD). Cigarette smoke, the major cause of COPD, contains very high concentrations of gaseous and soluble oxidants that can directly induce cell injury and death. Furthermore, particulate matter in cigarette smoke activates lung macrophages that subsequently attract neutrophils. Both neutrophils and macrophages from the lungs of cigarette smokers continuously release large amounts of superoxide and hydrogen peroxide through the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Once individuals with COPD stop smoking, the neutrophilic inflammation in the airways and lung parenchyma persists, as do the markers of oxidative stress. Several animal models of cigarette smoke-induced injury have provided evidence that various antioxidants may prevent inflammation and morphological changes associated with COPD however, evidence of benefit in patients is less abundant. Although oxidants can inactivate alpha-1 antitrypsin and other protective proteins, damage lung tissue, and increase mucus production, they also are essential for killing pathogens and resolving inflammation. This review will examine the pre-clinical and clinical evidence of a role for antioxidants in the therapy of patients with COPD.

Citation

Citation: Vézina FA, Cantin AM. Antioxidants and chronic obstructive pulmonary disease. J COPD F. 2018; 5(4): 277-288. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0133

Keywords

emphysema, inflammation, alpha-1antitrypsin, N-acetylcysteine, cigarette smoke

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Click to read Antioxidants and Chronic Obstructive Pulmonary Disease

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Author Affiliations

1. Department of Pharmaceutics, College of Pharmacy, University of Florida, Gainesville

Address correspondence to:

Sihong Song, PhD
Department of Pharmaceutics
College of Pharmacy University of Florida
Gainesville, FL 32610
Email: shsong@ufl.edu
Phone: 352-273-7867

Abstract

Autoimmune diseases are conditions caused by an over reactive immune system that attacks self-tissues and organs. Although the pathogenesis of autoimmune disease is complex and multi-factorial, inflammation is commonly involved. Therefore, anti-inflammatory therapies hold potential for the treatment of autoimmune diseases. However, long-term control of inflammation is challenging and most of the currently used drugs have side effects. Alpha-1 antitrypsin (AAT) is an anti-inflammatory protein with a well-known safety profile. The therapeutic potential of AAT has been tested in several autoimmune disease models. The first study using a recombinant adeno-associated viral (rAAV) vector showed that AAT gene transfer prevented the development of type 1 diabetes (T1D) in the non-obese diabetic (NOD) mouse model. Subsequent studies showed that treatment with AAT protein prevented and reversed type 1 diabetes. The beneficial effects of AAT treatment have also been observed in other autoimmune disease models such as rheumatoid arthritis and systemic lupus erythematosus. This paper reviews the therapeutic application of AAT and discusses possible mechanisms of action in various autoimmune diseases.

Citation

Citation: Song S. Alpha-1 antitrypsin therapy for autoimmune disorders. J COPD F. 2018; 5(4): 289-301. doi: http://doi.org/10.15326/jcopdf.5.4.2018.0131

Keywords

chronic obstructive pulmonary disease, alpha-1 antitrypsin deficiency, alpha-1 antitrypsin, autoimmunity, type 1 diabetes, rheumatoid arthritis, systemic lupus erythematosus

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Click to read Alpha-1 Antitrypsin Therapy for Autoimmune Disorders

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Author Affiliations

1. Center for Regenerative Medicine of Boston University and Boston Medical Center, Boston, Massachusetts

Address correspondence to:

Andrew A. Wilson, MD
Center for Regenerative Medicine
Boston University and Boston Medical Center
670 Albany St., 2nd Floor
Boston, MA 02118
Tel: 617-414-3282
Fax: 617-536-8093
Email: awilson@bu.edu

Abstract

PIZZ alpha-1 antitrypsin deficiency (AATD) is an autosomal recessive disease affecting approximately 100,000 individuals in the United States and one of the most common hereditary causes of liver disease.¹ The most common form of the disease results from a single base pair mutation (Glu342Lys), known as the “Z” mutation, that encodes a mutant protein (Z alpha-1 antritypsin [AAT]) that is prone to misfolding and is retained in the endoplasmic reticulum (ER) rather than appropriately secreted. Some of the retained mutant protein attains an unusual aggregated or polymerized conformation. Retained polymeric ZAAT aggregates are hepatotoxic and lead to downstream liver disease in a subset of PiZZ neonates and adults through a gain-of-function mechanism. PiZZ individuals are likewise highly predisposed to developing chronic obstructive pulmonary disease (COPD)/emphysema as a result of low circulating levels of AAT protein and associated protease-antiprotease imbalance. Much of our understanding of the molecular pathogenesis of AATD is based on studies employing either transgenic mice that express the mutant human Z allele or immortalized cell lines transduced to overexpress ZAAT. While they have been quite informative, these models fail to capture the patient-to-patient variability in disease phenotype that clinicians observe in their AATD patients, raising the question of whether alternative models might provide new insight. Induced pluripotent stem cells (iPSCs), first described in 2006, have the capacity to differentiate into a broad array of cell types from all 3 germ layers, including hepatocytes. Disease-specific iPSCs have been derived from patients with a variety of monogenic disorders and have been found to faithfully recapitulate features of such diseases as spinal muscular atrophy, familial dysautonomia, Rett syndrome, polycythemia vera, type 1A glycogen storage disease, familial hypercholesterolemia, long QT syndrome, and others. This discussion reviews the potential applications of iPSCs for understanding AATD-associated liver disease as well as for development of potential therapeutic strategies.

Citation

Citation: Kaserman JE, Wilson AA. Patient-derived induced pluripotent stem cells for alpha-1 antitrypsin deficiency disease modeling and therapeutic discovery. J COPD F. 2018; 5(4): 258-266. doi: http://doi.org/10.15326/jcopdf.5.4.2017.0179

Keywords

alpha-1 antitrypsin deficiency, induced pluripotent stem cells, protein misfolding

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Click to read Patient-Derived Induced Pluripotent Stem Cells for Alpha-1 Antitrypsin Deficiency Disease Modeling and Therapeutic Discovery

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Author Affiliations

1. Department of Clinical Biochemistry and Pharmacology, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel

Address correspondence to:

Eli C. Lewis, PhD
Department of Clinical Biochemistry & Pharmacology
Faculty of Health Sciences
Ben-Gurion University of the Negev
POB 151, Be’er-Sheva 8410501, Israel
Phone: ++972-8-6244-574
e-mail: lewis@bgu.ac.il

Abstract

Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder which most commonly manifests as pulmonary emphysema. Accordingly, alpha-1 antitrypsin (AAT) augmentation therapy aims to reduce the progression of emphysema, as achieved by life-long weekly slow-drip infusions of plasma-derived affinity-purified human AAT. However, not all AATD patients will receive this therapy, due to either lack of medical coverage or low patient compliance. To circumvent these limitations, attempts are being made to develop lung-directed therapies, including inhaled AAT and locally-delivered AAT gene therapy. Lung transplantation is also an ultimate therapy option. Although less common, AATD patients also present with disease manifestations that extend beyond the lung, including vasculitis, diabetes and panniculitis, and appear to experience longer and more frequent hospitalization times and more frequent pneumonia bouts. In the past decade, new mechanism-based clinical indications for AAT therapy have surfaced, depicting a safe, anti-inflammatory, immunomodulatory and tissue-protective agent. Introduced to non-AATD individuals, AAT appears to provide relief from steroid-refractory graft-versus-host disease, from bacterial infections in cystic fibrosis and from autoimmune diabetes; preclinical studies show benefit also in multiple sclerosis, ulcerative colitis, rheumatoid arthritis, acute myocardial infarction and stroke, as well as ischemia-reperfusion injury and aberrant wound healing processes. While the current augmentation therapy is targeted towards treatment of emphysema, it is suggested that AATD patients may benefit from AAT augmentation therapy geared towards extrapulmonary pathologies as well. Thus, development of mechanism-based, context-specific AAT augmentation therapy protocols is encouraged. In the current review, we will discuss extrapulmonary manifestations of AATD and the potential of AAT augmentation therapy for these conditions.

Citation

Citation: Baranovski BM, Schuster R, Nisim O, Brami I, Lewis EC. Alpha-1 antitrypsin substitution for extrapulmonary conditions in alpha-1 antitrypsin deficient patients. J COPD F. 2018; 5(4): 267-276. doi: http://doi.org/10.15326/jcopdf.5.4.2017.0161

Keywords

inflammation, diabetes, autoimmunity, bone-marrow transplantation, cell survival, immune system, leukemia, organ transplantation, ulcerative colitis, wound healing

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Click to read Alpha-1 Antitrypsin Substitution for Extrapulmonary Conditions in Alpha-1 Antitrypsin Deficient Patients

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Author Affiliations

1. Department of Genetic Medicine, Weill Cornell Medical College, New York, New York

*  KMS and DS contributed equally to this review

Address correspondence to:

Katie M. Stiles, PhD or
Dolan Sondhi, PhD
Department of Genetic Medicine
Weill Cornell Medical College
1300 York Avenue, Box 164
New York, New York 10065
Phone: (646) 962-4363
Fax: (646) 962-0220
E-mail: geneticmedicine@med.cornell.edu

Abstract

Alpha-1 antitrypsin deficiency (AATD) manifests primarily as early-onset emphysema caused by the destruction of the lung by neutrophil elastase due to low amounts of the serine protease inhibitor alpha-1 antitrypsin (AAT). The current therapy involves weekly intravenous infusions of AAT-derived from pooled human plasma that is efficacious, yet costly. Gene therapy applications designed to provide constant levels of the AAT protein are currently under development. The challenge is for gene therapy to provide sufficient amounts of AAT to normalize the inhibitor level and anti-neutrophil elastase capacity in the lung. One strategy involves administration of an adeno-associated virus (AAV) gene therapy vector to the pleural space providing both local and systemic production of AAT to reach consistent therapeutic levels. This review focuses on the strategy, advantages, challenges, and updates for intrapleural administration of gene therapy vectors for the treatment of AATD.

Citation

Citation: Stiles KM, Sondhi D, Kaminsky SM, De BP, Rosenberg JB, Crystal RG. Intrapleural gene therapy for alpha-1 antitrypsin deficiency-related lung disease. J COPD F. 2018; 5(4): 244-257. doi: http://doi.org/10.15326/jcopdf.5.4.2017.0160

Keywords

alpha-1 antitrypsin deficiency, gene therapy

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Click to read Intrapleural Gene Therapy for Alpha-1 Antitrypsin Deficiency-Related Lung Disease

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Author Affiliations

1. UCL Respiratory, Division of Medicine, University College London, United Kingdom

Address correspondence to:

David Lomas, MD, PhD
UCL Respiratory, Division of Medicine
Rayne Building
University College London
WC1E 6BN
London, UK
Email: d.lomas@ucl.ac.uk

Abstract

Alpha-1antitrypsin deficiency (AATD) results from the intracellular polymerization and retention of mutant alpha-1antitrypsin (AAT) within the endoplasmic reticulum of hepatocytes. This causes cirrhosis whilst the deficiency of circulating AAT predisposes to early onset emphysema. This is an exciting time for researchers in the field with the development of novel therapies based on understanding the pathobiology of disease. I review here augmentation therapy to prevent the progression of lung disease and a range of approaches to treat the liver disease associated with the accumulation of mutant AAT: modifying proteostasis networks that are activated by Z AAT polymers, stimulating autophagy, small interfering RNA and small molecules to block intracellular polymerization, and stem cell technology to correct the genetic defect that underlies AATD.

Citation

Citation: Lomas DA. New therapeutic targets for alpha-1 antitrypsin deficiency. J COPD F. 2018; 5(4): 233-243. doi: http://doi.org/10.15326/jcopdf.5.4.2017.0165

Keywords

emphysema, alpha-1 antitrypsin deficiency, cirrhosis, conformational disease, small molecules, siRNA, proteostasis, autophagy

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Click to read New Therapeutic Targets for Alpha-1 Antitrypsin Deficiency

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Author Affiliations

1. Denver, Colorado

Address correspondence to:

Ron Balkissoon, MD, MSc, DIH, FRCPC
balkissoonr@njhealth.org

Abstract

This article does not contain an abstract.

Citation

Citation: Balkissoon R.Journal club. Impact of the IMPACT Trial. J COPD F. 2018; 5(3): 221-227. doi: http://doi.org/10.15326/jcopdf.5.3.2018.0150

Keywords

There are no keywords for this article.

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Click to read Impact of the IMPACT Trial

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Author Affiliations

1. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine, Temple University Health System, Philadelphia, Pennsylvania
2. Department of Epidemiology, Colorado School of Public Health, Anschutz Medical Campus, University of Colorado, Aurora
3. Division of Pulmonary, Allergy and Critical Care Medicine, University of Alabama, Birmingham
4. Division of Pulmonary and Critical Care Medicine, Morehouse School of Medicine, Atlanta, Georgia

Address correspondence to:

A. James Mamary, MD
Temple Lung Center 745
Parkinson Pavilion 3401
North Broad Street Philadelphia Pa, 19140
Phone: 215-707-3332
Email: albert.mamary@tuhs.temple.edu

Abstract

The COPD Genetic Epidemiology (COPDGene®) study provides a rich cross-sectional dataset of patients with substantial tobacco smoke exposure, varied by race, gender, chronic obstructive pulmonary disease (COPD) diagnosis, and disease. We aimed to determine the influence of race, gender and Global initiative for chronic Obstructive Lung Disease (GOLD) stage on prevalence of prior COPD diagnosis at COPDGene® enrollment. Data from the complete phase 1 cohort of 10,192 participants were analyzed. Participants were non-Hispanic white and African-American, ≥45 years of age with a minimum of 10 pack years of cigarette smoking. Characterization upon enrollment included spirometry, demographics and history of COPD diagnosis determined by questionnaire. We evaluated the effects of race and gender on the likelihood of prior diagnosis of COPD and the interaction of race and GOLD stage, and gender and GOLD stage, as determined at study enrollment, on likelihood of prior diagnosis of COPD. We evaluated the 3-way interaction of race, gender and GOLD stage on prior diagnosis. African-Americans had higher odds of not having a prior COPD diagnosis at all GOLD stages of airflow obstruction versus non-Hispanic whites (p<0.0001). Women had higher odds of having a prior COPD diagnosis at all GOLD stages versus men (p<0.0001). Three-way interaction of race, gender and GOLD stage was not significant. African-Americans were less likely to have prior COPD regardless of the severity of airflow obstruction determined at study enrollment. Women were more likely to have a prior COPD diagnosis regardless of the severity of measured airflow obstruction. Race and gender are associated with significant disparities in COPD diagnosis.

Citation

Citation: Mamary AJ, Stewart JI, Kinney GL, et al for the COPDGene investigators. Race and gender disparities are evident in COPD underdiagnoses across all severities of measured airflow obstruction. J COPD F. 2018; 5(3): 177-184. doi: http://doi.org/10.15326/jcopdf.5.3.2017.0145

Keywords

spirometry, diagnosis, asthma, tobacco, epidemiology, smokers

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Click to read Race and Gender Disparities are Evident in COPD Underdiagnoses Across all Severities of Measured Airflow Obstruction

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Author Affiliations

1. Division of Pulmonary and Critical Care Medicine, Department of Medicine, Intermountain Medical Center, Murray, Utah
2. Division of Respiratory, Critical Care, and Sleep Medicine, Department of Medicine, University of Utah, Salt Lake City
3. Office of Research, Intermountain Healthcare, Salt Lake City, Utah
4. Homer Warner Center for Informatics Research, Murray, Utah
5. Section of General Internal Medicine, Department of Medicine, University of Chicago Medicine, Chicago, Illinois
6. Kaiser Permanente Center for Health Research – Northwest, Portland, Oregon
7. Division of Pulmonary and Critical Care Medicine, Oregon Health and Science University, Portland
8. Division of Pulmonary and Critical Care Medicine, San Francisco Veterans Affairs Medical Center, San Francisco, California
9. Division of Pulmonary, Critical Care Allergy, Immunology and Sleep Medicine, Department of Medicine, University of California, San Francisco

Address correspondence to:

Denitza P. Blagev, MD
Intermountain Medical Center
Heart Lung Building, 6^th Floor
5121 South Cottonwood Street
Murray, UT 84157
Email: denitza.blagev@imail.org
denitza.blagev@gmail.com
Phone: (801) 507 4695

Abstract

Rationale: Although chronic obstructive pulmonary disease (COPD) exacerbation frequency is stable in research cohorts, whether severe COPD exacerbation frequency can be used to identify patients at high risk for future severe COPD exacerbations and/or mortality is unknown.

Methods: Severe COPD exacerbation frequency stability was determined in 3 distinct clinical cohorts. A total of 17,450 patients with COPD in Intermountain Healthcare were categorized based on the number of severe COPD exacerbations per year. We determined whether exacerbation frequency was stable and whether it predicted mortality. These findings were validated in 83,134 patients from the U.S. Veterans Affairs (VA) nationwide health care system and 3326 patients from the University of Chicago Medicine health system.

Results: In the Intermountain Healthcare cohort, the majority (84%, 14,706 patients) had no exacerbations in 2009 and were likely to remain non-exacerbators with a significantly lower 6-year mortality compared with frequent exacerbators (2 or more exacerbations per year) (25% versus 57%, p<0.001). Similar findings were noted in the VA health system and the University of Chicago Medicine health system. Non-exacerbators were likely to remain non-exacerbators with the lowest overall mortality. In all cohorts, frequent exacerbator was not a stable phenotype until patients had at least 2 consecutive years of frequent exacerbations. COPD exacerbation frequency predicted any cause mortality.

Conclusions: In clinical datasets across different organizations, severe COPD exacerbation frequency was stable after at least 2 consecutive years of frequent exacerbations. Thus, severe COPD exacerbation frequency identifies patients across a health care system at high risk for future COPD-related health care utilization and overall mortality.

Citation

Citation: Blagev DP, Collingridge DS, Rea S, et al. Stability of frequency of severe chronic obstructive pulmonary disease exacerbations and health care utilization in clinical populations. J COPD F. 2018; 5(3): 208-220. doi: http://doi.org/10.15326/jcopdf.5.3.2017.0183

Keywords

chronic obstructive pulmonary disease, COPD exacerbation, health care utilization, stability

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Click to read Stability of Frequency of Severe Chronic Obstructive Pulmonary Disease Exacerbations and Health Care Utilization in Clinical Populations

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Author Affiliations

1. Pulmonary Research Institute of Southeast Michigan, Farmington Hills
2. Clinical Research Institute of Southern Oregon, PC, Medford
3. University of North Carolina School of Medicine, Chapel Hill
4. Sunovion Pharmaceuticals Inc., Marlborough, Massachusetts
5. Abilytic, Inc., Shrewsbury, Massachusetts

Address correspondence to:

Gary T. Ferguson, MD
Pulmonary Research Institute of Southeast Michigan
29255 West 10 Mile Road, Suite A
Farmington Hills, MI 48336
Tel: (248) 478-6561
Fax: (248) 478-6908
Email: garytferguson@msn.com

Abstract

Background: Symptoms of chronic obstructive pulmonary disease (COPD) may diminish patients’ health-related quality of life (HRQoL). We report effects of Longhala™ Magnair™ (glycopyrrolate) Inhalation Solution, a drug/device combination of the long-acting antimuscarinic glycopyrrolate administered using the eFlow® closed system (eFlow CS) nebulizer, on HRQoL from the Glycopyrrolate for Obstructive Lung Disease Via Electronic Nebulizer (GOLDEN) clinical studies.

Methods: Data consisted of a pooled analysis of 2 phase 3, 12-week efficacy studies (GOLDEN-3 and -4) of glycopyrrolate/eFlow CS (25 or 50 mcg twice daily [BID]) versus placebo, and a 48-week, open-label safety study (GOLDEN-5) of glycopyrrolate/eFlow CS 50 mcg BID versus tiotropium 18 mcg once daily in patients with moderate to very severe COPD. Change from baseline in HRQoL was measured via the St George’s Respiratory Questionnaire (SGRQ). Results are provided as mean changes in SGRQ Total score and as response analysis (≥4-point improvement [responder], no change, and ≥4-point worsening in Total score) using analysis of covariance or logistic regression, as applicable.

Results: A total of 1293 patients were evaluated from GOLDEN-3 and -4 and 1086 from GOLDEN-5. Glycopyrrolate/eFlow CS significantly improved SGRQ Total and component scores. The percentage of SGRQ responders in pooled GOLDEN-3/4 was 46.8% for glycopyrrolate/eFlow CS 25 mcg, 41.7% for glycopyrrolate/eFlow CS 50 mcg, and 34.5% for placebo. SGRQ Total and component score improvements were similar between glycopyrrolate/eFlow CS and tiotropium in GOLDEN-5.

Conclusions: The drug/device combination of glycopyrrolate/eFlow CS significantly improved HRQoL, as measured by the SGRQ, offering a potential maintenance treatment option in patients with moderate to very severe COPD.

ClinicalTrials.gov: NCT02347761, NCT02347774, NCT02276222

Citation

Citation: Ferguson GT, Kerwin EM, Donohue JF, et al. Health-related quality of life improvements in moderate to very severe chronic obstructive pulmonary disease patients on nebulized glycopyrrolate: evidence from the GOLDEN studies. J COPD F. 2018; 5(3): 193-207. doi: http://doi.org/10.15326/jcopdf.5.3.2017.0178

Keywords

COPD, chronic obstructive pulmonary disease, health status, long-acting muscarinic antagonist, health-related quality of life, nebulizer, HRQoL

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Click to read Health-Related Quality of Life Improvements in Moderate to Very Severe Chronic Obstructive Pulmonary Disease Patients on Nebulized Glycopyrrolate: Evidence from the GOLDEN Studies

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Author Affiliations

1. Department of Internal Medicine, Danbury Hospital, Western Connecticut Health Network, Danbury
2. Department of Research & Innovation, Danbury Hospital, Western Connecticut Health Network, Danbury
3. Section of Pulmonary Medicine, Department of Internal Medicine, Danbury Hospital, Western Connecticut Health Network, Danbury

Address correspondence to:

John Chronakos, MD
33 Germantown Road
Danbury, CT 06810
E-mail: john.chronakos@gmail.com
Phone: (203)739-6805

Abstract

Acute exacerbation of chronic obstructive pulmonary disease (AECOPD) is one of the leading causes of hospitalization in the United States. Prior investigations suggest clinical and physiological parameters are important determinants for AECOPD readmissions. Strategies aimed at addressing these factors have not resulted in a major reduction of readmissions. We compared patients readmitted after an index AECOPD admission with non-readmitted patients. Patients’ age, gender, body mass index, comorbidities (obstructive sleep apnea, chronic hypercapnia, congestive heart failure, lung cancer, pulmonary arterial hypertension, pneumonia, interstitial lung disease, atrial fibrillation, musculoskeletal disorders, cognitive disorders, and anxiety disorders), substance abuse and smoking status were assessed. Some 272 patients were included: 20 patients were readmitted within 30 days of their index hospitalization; 252 patients were not readmitted within 30 days of their index admission. Readmitted patients were significantly more likely to have pneumonia than non-readmitted patients (30.0% versus 13.1%, p<0.05). No statistically significant difference was seen with respect to other clinical comorbidities. Patients readmitted within 30 days were significantly more likely than non-readmitted patients to have safety issues at home (80.0% versus. 39.3%, p<0.001), anxiety (60.0% versus 29.8%, p<0.01), and lack of transportation (35.0% versus 15.5%, p<0.05). Implementation of a comprehensive care management program (CCMP) was associated with a reduction in readmissions from 21.5% to 13.6% (p<0.01, 95% confidence interval [CI] 2.08-12.45). A CCMP can reduce readmissions through attention to social variables, optimization of in-hospital care, improved coordination of pre- and post-discharge, a system to better identify problems after discharge, and an office setup that accommodates same-day visits.

Citation

Citation: Euceda G, Kong W-T, Kapoor A^, Dilauro P, Ogunnaike R, Chronakos J. The effects of a comprehensive care management program on readmission rates after acute exacerbation of COPD at a community-based academic hospital. J COPD F. 2018; 5(3): 185-192. doi: http://doi.org/10.15326/jcopdf.5.3.2017.0177

Keywords

population health, rehospitalization prevention, risk factors for rehospitalization

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Click to read The Effects of a Comprehensive Care Management Program on Readmission Rates After Acute Exacerbation of COPD at a Community-Based Academic Hospital

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Author Affiliations

1. Emeritus Professor of Medicine, Geisel School of Medicine at Dartmouth, Hanover, New Hampshire

2. Valley Regional Hospital, Claremont, New Hampshire
3. Medical Dynamics, New York, New York
4. University of Louisville, Louisville, Kentucky
5. Rockpile Strategies LLC, LaGrange, Illinois

Address correspondence to:

Donald A. Mahler, MD
Valley Regional Hospital
243 Elm Street
Claremont, NH 03743
Phone: 603 277-0383
Email: mahlerdonald@gmail.com

Abstract

Background: Little is known about patients’ use of the internet to search for information about chronic obstructive pulmonary disease (COPD) and their perspectives on disease content on websites.

Objectives: Todetermine the interests and behavior of patients with COPD who search the internet for disease information and to assess their perspectives about 2 COPD educational websites.

Methods: Individuals with COPD who had registered for a consumer panel were invited electronically to participate in a survey which included general use of the internet, online health behaviors about COPD, and assessment of 2COPD educational websites.

Results: A total of 445 respondents completed the survey in 23 ± 12 minutes (72% response rate). A total of 95% reported that physicians were the primary source of information about COPD followed by internet searches about the disease (76%). The 3 major information priorities were “symptom control” (82%), “how COPD is affecting my body” (60%), and “treatments that might work better for me” (59%). Overall ratings (range, 1 – 10) were 7.4 ± 1.5 for the American Lung Association and 6.8 ±1.8 for the COPD Foundation websites. Ratings by respondents were higher for all 5 impression attributes and for 8 of 9 content attributes on the American Lung Association website compared with the COPD Foundation website.

Conclusions: This report describes, for the first time, information priorities of patients with COPD about their disease and their assessment of 2 educational websites. Our survey results can be used by health care professionals to recommend online resources to their patients.

Citation

Citation: Mahler DA, Cerasoli F, Della L, Rudzinski M. Internet health behaviors of patients with chronic obstructive pulmonary disease and assessment of two disease websites. J COPD F. 2018; 5(3): 158-166. doi: http://doi.org/10.15326/jcopdf.5.3.2017.0173

Keywords

COPD, exacerbations, internet.chronic obstructive pulmonary disease, COPD websites, breathlessness

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Click to read Internet Health Behaviors of Patients with Chronic Obstructive Pulmonary Disease and Assessment of Two Disease Websites

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Author Affiliations

1. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania
2. Division of Biostatistics and Bioinformatics, Bloomberg School of Health, Johns Hopkins University, Baltimore, Maryland
3. Division of Pulmonary and Critical Care Medicine, School of Medicine, Louisiana State University, New Orleans
4. Channing Laboratory, Brigham and Women’s Hospital and Division of Pulmonary and Critical Care Medicine, Harvard Medical School, Boston, Massachusetts
5. Division of Pulmonary and Critical Care Medicine, School of Medicine, University of California- San Diego
6. Division of Pulmonary and Critical Care Medicine, School of Medicine, University of Colorado, Denver
7. Members of the LOTT Research Group and their affiliations are listed in the Acknowledgements section of this article.

Address correspondence to:

Erin R. Narewski, DO
Department of Thoracic Medicine and Surgery
Lewis Katz School of Medicine
Temple University
3401 North Broad Street
Philadelphia, PA 19140
Erin.Narewski@tuhs.temple.edu

Abstract

Background: Chronic obstructive pulmonary disease (COPD) patients enrolled into the Long-term Oxygen Treatment Trial had hypoxemia at rest, hypoxemia on exertion, or hypoxemia both at rest and on exertion. We hypothesized that patients with different patterns of hypoxemia may have significant differences in clinical features.

Methods: All patients had COPD and oxygen saturation measured by pulse oximetry (blood oxygenation [SpO₂]) at rest and during the 6-minute walk test (6MWT). Hypoxemia at rest was defined as resting SpO₂ between 89-93%. SpO₂ < 90% for at least 10 seconds and ³ 80% for at least 5 minutes during ambulation characterized hypoxemia on exertion. Severe exercise hypoxemia (< 80% for > 1 minute) was exclusionary.

Results: Of 738 patients studied, 133 (18.0%) had mild-moderate hypoxemia at rest only, 319 (43.2%) had hypoxemia on exertion only, and 286 (38.8%) had hypoxemia at both rest and exertion. Patients with hypoxemia at rest only were more likely to be current smokers, had higher body mass index (BMI) and a higher incidence of self-reported diabetes. Patients with hypoxemia on exertion only were more severely obstructed compared to the other groups. General and disease-specific quality of life scores were similarly impaired in all groups. Quality of well-being scores were more impaired in those with hypoxemia at rest only.

Conclusions: COPD patients with mild-moderate hypoxemia have distinct clinical characteristics based on the pattern of oxygen desaturation at rest and with exertion.

Citation

Citation: Narewski ER, Blackford AL, Lammi MR, et al; for the Long-term Oxygen Treatment Trial Research Group. Clinical differences in COPD patients with variable patterns of hypoxemia. J COPD F. 2018; 5(3): 167-176. doi: http://doi.org/10.15326/jcopdf.5.3.2017.0175

Keywords

COPD, chronic obstructive pulmonary disease, long-term oxygen treatment, Long-term Oxygen Treatment Trial, LOTT

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Click to read Clinical Differences in COPD Patients with Variable Patterns of Hypoxemia

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Author Affiliations

1. Denver, Colorado

Address correspondence to:

Ron Balkissoon, MD, MSc, DIH, FRCPC
balkissoonr@njhealth.org

Abstract

This article does not contain an abstract.

Citation

Citation: Balkissoon R. Journal club. Stem cell therapy for COPD: where are we? J COPD F. 2018; 5(2): 148-153. doi: http://doi.org/10.15326/jcopdf.5.2.2018.0140

Keywords

There are no keywords for this article.

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Click to read Stem Cell Therapy for COPD: Where are we?

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Author Affiliations

1. Department of Radiology, Mount Sinai Medical Center, New York, New York
2. Columbia University Medical Center, New York, New York

Address correspondence to:

Mary Salvatore, MD
Department of Radiology
Mount Sinai Medical Center
1 Gustave L. Levy Place
New York, NY 10029
mary.salvatore@mountsinai.org

Abstract

This article does not contain an abstract.

Citation

Citation: Salvatore M, Kwon K, Steiner RM. Images in COPD: combined pulmonary fibrosis and emphysema. J COPD F. 2018; 5(2): 154-157. doi: http://doi.org/10.15326/jcopdf.5.2.2018.0137

Keywords

COPD, chronic obstructive pulmonary disease, combined pulmonary fibrosis and emphysema, CPFE, idiopathic pulmonary fibrosis, IPF

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Click to read Combined Pulmonary Fibrosis and Emphysema

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Author Affiliations

1. St. Francis Hospital and University Medical Research LLC

Address correspondence to:

Nicholas Gross, MD, PhD
Grossnicholas1@gmail.com

Abstract

This article does not contain an abstract.

Citation

Citation: Gross N. COPD pipeline XXXVII. J COPD F. 2018; 5(2): 144-147. doi: http://doi.org/10.15326/jcopdf.5.2.2018.0136

Keywords

INS1007, Pioneer, doxofylline, QVA, Gala treatment, Nemiralisib, CHI3L1, YKL-40

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Click to read The COPD Pipeline XXXVIII

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Author Affiliations

1. Pneumology and Critical Care Medicine, Thoraxklinik at University of Heidelberg, Heidelberg, Germany
2. Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany

Address correspondence to:

Daniela Gompelmann, MD
Phone: 004962213968087
Email: daniela.gompelmann@med.uni-heidelberg.de

Abstract

This article does not contain an abstract.

Citation

Citation: Gompelmann D. Editorial: Hope for patients with homogeneous emphysema? J COPD F. 2018; 5(2): 84-86. doi: http://doi.org/10.15326/jcopdf.5.2.2018.0135

Keywords

COPD, chronic obstructive pulmonary disease, lung volume reduction surgery, endobronchial coils, homogeneous emphysema, LVRS

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Click to read Hope for Patients with Homogeneous Emphysema?

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Author Affiliations

1. Department of Biomedical Sciences, College of Medicine, Inha University, Incheon, Republic of Korea
2. Channing Division of Network Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
3. Division of Pulmonary and Critical Care Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
4. Department of Medicine, National Jewish Health, Denver, Colorado
5. School of Public Health, University of Colorado, Denver
6. University of Pittsburgh, Pittsburgh, Pennsylvania

Address correspondence to:

Elizabeth A. Regan, MD
1400 Jackson St, K706
Denver, CO 80209
Email: ReganE@NJHealth.org
Phone: 303-398-1531
Fax: 303-270-2249

Abstract

Background: Adiponectin has been proposed as a biomarker of disease severity and progression in chronic obstructive pulmonary disease (COPD) and associated with spirometry-defined COPD and with computed tomography (CT)-measured emphysema. Increased adiponectin plays a role in other diseases including diabetes/metabolic syndrome, cardiovascular disease and osteoporosis. Previous studies of adiponectin and COPD have not assessed the relationship of adiponectin to airway disease in smokers and have not evaluated the effect of other comorbid diseases on the relationship of adiponectin and lung disease. We postulated that adiponectin levels would associate with both airway disease and emphysema in smokers with and without COPD, and further postulated that body composition and the comorbid diseases of osteoporosis, cardiovascular disease and diabetes might influence adiponectin levels.

Methods: Current and former smokers from the COPD Genetic Epidemiology study (COPDGene) (n= 424) were assigned to 4 groups based on CT lung characteristics and volumetric Bone Density (vBMD). Emphysema (% low attenuation area at -950) and airway disease (Wall area %) were used to assess smoking-related lung disease (SRLD). Group 1) Normal Lung with Normal vBMD; Group 2) Normal Lung and Osteoporosis; Group 3) SRLD with Normal vBMD; Group 4) SRLD with Osteoporosis. Cardiovascular disease (CVD), diabetes, C-reactive protein (CRP) and T-cadherin (soluble receptor for adiponectin) levels were defined for each group. Body composition was derived from chest CT. Multivariable regression assessed effects of emphysema, wall area %, bone density, comorbid diseases and other key factors on log adiponectin.

Results: Group 4, SRLD with Osteoporosis, had significantly higher adiponectin levels compared to other groups and the effect persisted in adjusted models. Systemic inflammation (by CRP) was associated with SRLD in Groups 3 and 4 but not with osteoporosis alone. In regression models, lower bone density and worse emphysema were associated with higher adiponectin. Airway disease was associated with higher adiponectin levels when T-cadherin was added to the model. Male gender, greater muscle and fat were associated with lower adiponectin.

Conclusions: Adiponectin is increased with both airway disease and emphysema in smokers. Bone density, and fat and muscle composition are all significant factors predicting adiponectin that should be considered when it is used as a biomarker of COPD. Increased adiponectin from chronic inflammation may play a role in the progression of bone loss in COPD and other lung diseases.

Citation

Citation: Suh YJ, McDonald M-LN, Washko GR, et al; for the COPDGene Investigators. Lung, fat and bone: increased adiponectin with the combination of smoking-related lung disease and osteoporosis. J COPD F. 2018; 5(2): 134-143. doi: http://doi.org/10.15326/jcopdf.5.2.2016.0174

Keywords

COPD, emphysema, body composition, adiponectin, osteoporosis, airway disease, smoking-related lung disease, QCT, volumetric BMD, bone mineral density, T cadherin, CDH13, C-reactive protein, systemic inflammation, muscle area, pectoralis, subcutaneous fat area, visceral fa

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Click to read Lung, Fat and Bone: Increased Adiponectin Associates with the Combination of Smoking-Related Lung Disease and Osteoporosis

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Author Affiliations

1. Graduate Medical Education, Arnot Ogden Medical Center, Elmira, New York

Address correspondence to:

James Tasch, DO
600 Roe Ave
Elmira, New York 14905
Phone: 607-737-4100
Email: james.tasch@steward.org

Abstract

Chronic obstructive pulmonary disease (COPD) currently affects more than 16 million Americans and it is estimated that roughly 100,000 Americans have undiagnosed, severe alpha-1 antitrypsin deficiency (AATD) (Chest. 2005;128[3]:1179-1186) (Chest. 2002;122[5]:1818-1829). Patients with AATD have an accelerated rate of decline of lung function caused by proteolytic enzymes. The morbidity associated with this inherited disorder is preventable due to the availability of augmentation therapy. Appropriate inpatient screening of patients with COPD for AATD is lacking and most screening is exclusively limited to outpatient pulmonary clinics. Between May 2016 and February 2017, genetic screening was completed on 54 individuals who were admitted with either a former diagnosis of COPD or active COPD exacerbation to Arnot Ogden Medical Center (AOMC) in Elmira, New York. The incorporation of inpatient genetic screening by resident physicians for AATD in COPD patients led to a high rate of screened and newly diagnosed AATD carriers with a variety of AATD genotypes. It is recommended that there should be an expansion of screening for AATD in hospitalized patients with COPD, regardless of age or smoking history.

Citation

Citation: Tasch JJ, McLaughlan AT, Nasir AA. A novel approach to screening for alpha-1 antitrypsin deficiency: inpatient testing at a teaching institution. J COPD F. 2018; 5(2): 106-110. doi: http://doi.org/10.15326/jcopdf.5.2.2017.0170

Keywords

COPD, chronic obstructive pulmonary disease, alpha-1 antitrypsin deficiency, inpatient, AAT, genetic screening, resident physician, AAT carriers

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Click to read A Novel Approach to Screening for Alpha-1 Antitrypsin Deficiency: Inpatient Testing at a Teaching Institution

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Author Affiliations

1. Department of Pulmonary and Critical Care, Wake Forest School of Medicine, Wake Forest Baptist Medical Center, Winston-Salem, North Carolina
2. Department of Internal Medicine, Wake Forest School of Medicine, Wake Forest Baptist Medical Center, Winston-Salem, North Carolina

Address correspondence to:

Chee Hong Loh, MD
Email: seanchloh@gmail.com
Phone: (336)716-4328

Abstract

Purpose: Objective documentation of airflow obstruction is often lacking inhospitalized patients treated for acute exacerbation of chronic obstructive pulmonary disease (AECOPD). The utility of spirometry performed in hospitalized patients to identify airflow obstruction, and thus a diagnosis of COPD, is unclear. Our aim was to compare inpatient spirometry, performed during an AECOPD, with outpatient spirometry.

Methods: A retrospective analysis of data from patients enrolled in an AECOPD care plan was performed. As part of the plan, patients underwent inpatient spirometry to establish a COPD diagnosis and outpatient clinic spirometry within 4 weeks of hospital discharge to confirm it. Data analyzed included forced expiratory volume in 1 second (FEV₁), forced vital capacity (FVC), slow vital capacity (SVC) and FEV₁/ vital capacity (VC). Obstruction was defined by FEV₁/VC<0.70.

Results: A total of 159 patients (mean age 63.2 +/- 10.5 years) had corresponding in- and outpatient spirometry. The median days between inpatient and outpatient spirometry was 12 (interquartile range [IQR] 9-16). Inpatient spirometry had a sensitivity of 94%, specificity of 24%, positive predictive value of 83% and negative predictive value of 53% for predicting outpatient obstruction. The area under curve for using inpatient spirometry was 0.82. The mean difference between inpatient and outpatient FEV₁ was 0.44 +/- 0.03 liters or 17.3 +/- 1.13 % predicted (p<0.0001) for FEV₁.

Conclusions: Inpatient spirometry accurately predicts outpatient airflow obstruction, thus providing an opportunity to identify patients admitted with suspected AECOPD who have no prior spirometric documentation.

Citation

Citation: Loh CH, Genese FA, Kannan KK, Lovings TM, Peters SP, Ohar JA. Spirometry in hospitalized patients with acute exacerbation of COPD accurately predicts post discharge airflow obstruction. J COPD F. 2018; 5(2): 124-133. doi: http://doi.org/10.15326/jcopdf.5.2.2017.0169

Keywords

COPD, chronic obstructive pulmonary disease, AECOPD, inpatient spirometry, COPD readmissions, acute exacerbations of COPD

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Click to read Spirometry in Hospitalized Patients with Acute Exacerbation of COPD Accurately Predicts Post Discharge Airflow Obstruction

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Author Affiliations

1. Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania
2. University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
3. Thoraxklinik, University of Heidelberg, Heidelberg, Germany
4. The National Institute for Health Research Respiratory Biomedical Research Unit, Royal Brompton & Harefield NHS Foundation Trust and Imperial College, London, United Kingdom
5. University Medical Center Groningen, University of Groningen, The Netherlands
6. Gartnaval General Hospital, Glasgow, Scotland
7. Klinikum Donaustauf, Donaustauf, Germany
8. Lungenklinik, Hemer, Germany
9. Campus Charité Mitte, Berlin, Germany
10. Service de Pneumologie Hôpital Maison Blanche, INSERM 903, Reims, France
11. Asklepios-Fachkliniken, Muenchen-Gauting, Germany
12. Krankenhaus von Roten Kreuz, Stuttgart, Germany
13. CHU de Strasbourg – NHC, Strasbourg, France
14. FHU OncoAge Côte d’Azur University, Nice, France
15. Department of Internal Medicine II-Pneumology, University Hospital, Teubingen, Germany

Address correspondence to:

Nathaniel Marchetti, D.O.
Associate Professor of Medicine
Department of Thoracic Medicine and Surgery
Lewis Katz School of Medicine at Temple University
745 Parkinson Pavilion
3401 North Broad Street
Philadelphia, Pa 19140
Phone: 215-707-9929
Email: nathaniel.marchetti@tuhs.temple.edu

Abstract

Rationale: Bronchoscopic lung volume reduction utilizing shape-memory nitinol endobronchial coils (EBC) may be safer and more effective in severely hyperinflated homogeneous emphysema compared to medical therapy or lung volume reduction surgery (LVRS).

Methods: The effect of bilateral EBC in patients with homogeneous emphysema on spirometry, lung volumes and survival was compared to patients with homogeneous emphysema randomized in the National Emphysema Treatment Trial (NETT) to LVRS or medical therapy. NETT participants were selected to match EBC participants in age, baseline spirometry, and gender. Outcomes were compared from baseline, at 6 and 12 months.

Results: There were no significant baseline differences in gender in the EBC, NETT-LVRS or medical treatment patients. At baseline no differences existed between EBC and NETT-LVRS patients in forced expiratory volume in 1 second ( FEV₁) or total lung capacity (TLC) %-predicted; residual volume (RV) and diffusing capacity of the lung for carbon monoxide (DLco) %-predicted were higher in the EBC group compared to NETT-LVRS (p < 0.001). Compared to the medical treatment group, EBC produced greater improvements in FEV₁ and RV but not TLC at 6 months. FEV₁ and RV in the EBC group remained significantly improved at 12-months compared to the medical treatment group. While all 3 therapies improved quality of life, survival at 12 months with EBC or medical therapy was greater than NETT-LVRS.

Conclusion: EBC may be a potential therapeutic option in patients with severe homogeneous emphysema and hyperinflation who are already receiving optimal medical treatment.

Citation

Citation: Marchetti N, Kaufman T, Chandra D, et al. Endobronchial coils versus lung volume reduction surgery or medical therapy for treatment of advanced homogenous emphysema. J COPD F. 2018; 5(2): 87-96. doi: http://doi.org/10.15326/jcopdf.5.2.2017.0134

Keywords

emphysema, lung volume reduction, lung volume reduction coils

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Click to read Endobronchial Coils Versus Lung Volume Reduction Surgery or Medical Therapy for Treatment of Advanced Homogenous Emphysema

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Author Affiliations

1. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania
2. Department of Internal Medicine, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania

Address correspondence to:

Nathaniel Gaeckle
Email: gaeckle@umn.edu
Phone: 612-805-8388

Abstract

Abstract:

Background: Poor dental health occurs in patients with chronic obstructive pulmonary disease (COPD); some evidence suggests that it may correlate with lower forced expiratory volume in 1 second (FEV₁) and 6-minute walk distance, and an increased rate of exacerbations. However, there is no data that examines how dental health may impact the daily respiratory symptoms that COPD patients experience. We prospectively studied indices of dental health and hygiene in patients with COPD and determined their impact on daily respiratory symptoms.

Methods: A total of 20 individuals with COPD (median [interquartile range (IQR)] % FEV₁ 37 [29-43]) and 10 healthy control individuals with no lung disease were recruited. Dental questionnaires, spirometry, and a dental examination were administered on their initial visit. COPD participants were given an electronic COPD daily diary to document peak expiratory flow and the presence and magnitude of daily breathlessness, cough, sputum production, and wheeze.

Results: Compared to healthy controls, COPD participants had less teeth (median 16.5 versus 28, p=0.0001), a trend to a higher plaque index (median 2.2 versus 1.7, p=0.15), and worse oral health-related quality of life (median Oral Health Impact Profile score 12.0 versus 4.5, p=0.02). A greater number of teeth correlated with higher percentage of days with cough (r=0.48, p<0.05) and wheeze (r=0.47, p<0.05).

Conclusion: Individuals with severe COPD have poor oral hygiene and oral health-related quality of life. In the setting of poor dentition, a greater number of teeth correlates with more daily respiratory symptoms. More teeth may create a larger reservoir for inflammatory proteins and pathogenic bacteria to be aspirated into the airways.

Citation

Citation: Gaeckle NT, Heyman B, Criner AJ, Criner GJ. Markers of dental health correlate with daily respiratory symptoms in COPD. J COPD F. 2018; 5(2): 97-105. doi: http://doi.org/10.15326/jcopdf.5.2.2017.0159

Keywords

COPD, chronic obstructive pulmonary disease, dental, plaque index, oral health, respiratory symptoms

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Click to read Markers of Dental Health Correlate with Daily Respiratory Symptoms in COPD

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Author Affiliations

1. Section of Pulmonary and Critical Care Medicine, Baylor College of Medicine, Houston, Texas
2. Icahn School of Medicine at Mount Sinai, New York, New York
3. University of Texas Health Science Center, San Antonio
4. South Texas Veterans Health Care System, San Antonio
5. American College of Chest Physicians, Glenview, Illinois
6. Texas State University, San Marcos
7. Indiana University Health, Bloomington
8. Geisel School of Medicine at Dartmouth, Hanover, New Hampshire
9. Wake Forest Health University Medical Center, Winston-Salem, North Carolina
10. Sunovion Pharmaceuticals Inc., Marlborough, Massachusetts

Address correspondence to:

Nicola A. Hanania, MD, MS
Baylor College of Medicine
1504 Taub Loop
Houston, TX 77030
E-mail: hanania@bcm.edu
Phone: 713-873-3454

Abstract

Background: Inhaled medications form the foundation of pharmacologic treatment for chronic obstructive pulmonary disease (COPD).The Delivery Makes a Difference (DMaD) project was conducted to better understand health care provider (HCP) and patient perspectives about the role of inhalation delivery devices in COPD, and to examine the nature of educational efforts between HCPs and patients on proper device technique.

Methods: Data were derived from 2 original quantitative, web-based, descriptive, cross-sectional surveys distributed to HCPs who manage COPD (n=513) and patients with COPD (n=499) in the United States. Descriptive statistics were used to assess data across important demographic variables. Inferential statistics were used to assess differences in attitudinal, descriptive, and behavioral measures that were cross-tabulated with demographic data.

Results: When prescribing medication for newly diagnosed patients with stable or unstable COPD, only 37% of HCPs considered type of device to be highly important, with only 45% of HCPs assessing device technique in every newly diagnosed patient. Patients with COPD were also relatively unconcerned with proper device technique (64% never concerned), regardless of their COPD severity. Although patients did not identify education as a significant impediment to proper device use, they reported inconsistent educational experiences.

Conclusions: We found that HCPs and patients prioritize medication over device when selecting treatments, showing limited concerns about proper device use. These results highlight the need to coordinate professional education with patient-directed educational efforts to further promote proper device selection and use in COPD management.

Citation

Citation: Hanania NA, Braman S, Adams SG, et al. The role of inhalation delivery devices in COPD: Perspectives of patients and health care providers. J COPD F. 2018; 5(2): 111-123. doi: http://doi.org/10.15326/jcopdf.5.2.2017.0168

Keywords

COPD, inhalation therapy, inhalation device

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Click to read The Role of Inhalation Delivery Devices in COPD: Perspectives of Patients and Health Care Providers

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Author Affiliations

1. Denver, Colorado

Address correspondence to:

Ron Balkissoon, MD, MSc, DIH, FRCPC
balkissoonr@njhealth.org

Abstract

This article does not contain an abstract.

Citation

Citation: Balkissoon R. Journal club. New treatments options for COPD: How do we decide phenotypes, endotypes or treatable traits? J COPD F. 2018; 5(1): 72-80. doi: http://doi.org/10.15326/jcopdf.5.1.2018.0128

Keywords

There are no keywords for this article.

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Click to read New Treatment Options for COPD: How Do We Decide Phenotypes, Endotypes or Treatable Traits?

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Author Affiliations

1. Columbia University Medical Center, New York, New York

Address correspondence to:

Jay S. Leb, MD
Department of Radiology
Columbia University Medical Center
630 West 168^th Street, MC-28
New York, NY 10032
jl4763@cumc.columbia.edu

Abstract

This article does not contain an abstract.

Citation

Citation: Leb JS, D’Souza B, Steiner RM. Images in COPD: Marijuana lung. J COPD F. 2018; 5(1): 81-83. doi: http://doi.org/10.15326/jcopdf.5.1.2017.0180

Keywords

COPD, computed tomography, emphysema, chronic obstructive pulmonary disease, marijuana, marijuana lung

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Click to read Marijuana Lung

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Author Affiliations

1. St Francis Hospital and University Medical Research, LLC

Address correspondence to:

Nicholas Gross, MD, PhD
grossnicholas1@gmail.com

Abstract

This article does not contain an abstract.

Citation

Citation: Gross N. The COPD pipeline XXXVII. J COPD F. 2018; 5(1): 66-71. doi: http://doi.org/10.15326/jcopdf.5.1.2017.0184

Keywords

COPD, chronic obstructive pulmonary disease, GSK2269557, Mepolizumab, Danirixin, INS1007, drug pipeline, CHF6333, AZD8871, ZephyrEZB

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Click to read The COPD Pipeline XXXVII

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Author Affiliations

1. Pulmonary and Critical Care Medicine, Weill Cornell Medical College, Cornell University, New York, New York
2. Division of Pulmonary and Critical Care Medicine, New York Presbyterian/Weill Cornell Medical Center, New York, New York

Address correspondence to:

Michael S. Niederman, MD
1305 York Avenue, 10th Floor,
New York, NY 10065
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Citation

Citation: Niederman MS. Pneumonia complicating COPD: Are corticosteroids a help or a hindrance? J COPD F. 2018; 5(1): 1-4. doi: http://doi.org/10.15326/jcopdf.5.1.2018.0162

Keywords

COPD, chronic obstructive pulmonary disease, pneumonia, corticosteroids

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Click to read Pneumonia Complicating COPD: Are Corticosteroids a Help or a Hindrance?

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Author Affiliations

1. Pharmacy Services, Sanford Health, Fargo, North Dakota
2. North Dakota State University College of Health Professions, Fargo
3. Respiratory Care Services, Sanford Health, Fargo, North Dakota

Address correspondence to:

David D. Leedahl, PharmD
801 Broadway N, Rt 0204
Fargo, ND 58122
Phone: (701) 417-2309
E-mail: david.leedahl@sanfordhealth.org

Abstract

Background: Bronchodilator therapy is a foundation of chronic obstructive pulmonary disease (COPD) exacerbation treatment. Although international guidelines recommend short-acting formulations given multiple times per day, long-acting formulations have not been adequately evaluated. The objective of our study was to determine the effectiveness of umeclidinium-vilanterol (UME/VIL), long-acting beta2-agonist/long-acting muscarinic antagonist (LABA/LAMA) as a once-daily alternative for treating COPD exacerbations in hospitalized patients.

Methods: In this retrospective sequential period analysis, we reviewed electronic medical records of patients hospitalized for COPD exacerbations before (September 1, 2015 to February 29, 2016) and after (April 1, 2016 to September 30, 2016) incorporation of UME/VIL into our standard COPD protocol. Before implementation, patients received a daily anticholinergic plus twice-daily long-acting beta2-agonist therapy (tiotropium plus formoterol, n=65). After implementation, UME/VIL replaced the previous regimen (n=58). No other changes were made to the COPD protocol. The primary outcome was 30-day hospital readmission rate. Hospital length of stay, 30-day mortality, and cost of care were analyzed as secondary outcomes.

Results: A trend toward increased 30-day readmission rates in the post-intervention group (24.1% versus 10.8%, p=0.049) was no longer statistically significant after adjustment for severity of illness (based on case-mix index) and complications or comorbidities based on diagnosis-related group codes (adjusted odds ratio: 2.499; 95% confidence interval: 0.916-7.380; p=0.074).

Conclusion: After adjustment for potential confounders,the implementation of a LABA/LAMA combination product was not statistically associated with an increased 30-day readmission rate but was associated with lower cost of care.

Citation

Citation: Chapin TW, Mann MA, Brown GL, Leitheiser TL, Anderson B, Leedahl DD. Effectiveness of umeclidinium-vilanterol for protocolized management of chronic obstructive pulmonary disease exacerbation in hospitalized patients: A sequential period analysis. J COPD F. 2018; 5(1): 38-45. doi: http://doi.org/10.15326/jcopdf.5.1.2017.0163

Keywords

COPD, chronic obstructive pulmonary disease, exacerbations, tiotropium, bronchodilator, length of stay, umeclidinium, vilanterol, formoterol, hospital readmission, treatment protocol, respiratory therapy

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Click to read Effectiveness of Umeclidinium-Vilanterol for Protocolized Management of Chronic Obstructive Pulmonary Disease Exacerbation in Hospitalized Patients: A Sequential Period Analysis

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