19. Severe COPD and Lung Volume Reduction

We are extremely excited for the third and final installment in our Pulm PEEPs and ATS Clinical Problems Assembly collaborative series on COPD. Today, we are joined by Drs. Jessica Bon, Michael Lester, and Niru Putcha to discuss severe COPD management and the role of lung volume reduction procedures. If you missed the first two parts of our series, make sure to check out episode 1 on COPD diagnosis and initial management, and episode 2 on COPD exacerbations.

Meet our Guests

Jessica Bon is an Associate Professor of Medicine at the University of Pittsburgh School of Medicine where she is also the Program Director for the Pulmonary and Critical Care Medicine Fellowship. Her research and clinical interests focus on lung disease progression in COPD and she manages patients with difficult-to-treat and severe COPD and evaluates patients for lung volume reduction surgery. Jessica was the chair of the ATS Clinical Problems Assembly Programming Committee from 2021 – 2022.

Michael Lester is an Assistant Professor of Medicine at Vanderbilt University Medical Center. Michael’s interests span both pulmonary and critical care medicine. He specializes in patients with advanced COPD and evaluation for bronchoscopic lung volume reduction surgery.

Niru Putcha is an Associate Professor of Medicine at Johns Hopkins School of Medicine and is an integral member and mentor in the Obstructive Lung Disease Group. Her research and clinical interests focus on the role of comorbidities on clinical outcomes in individuals with COPD. She also manages patients with difficult-to-treat and severe COPD and evaluates patients for lung volume reduction surgery. Niru is also the new chair of the ATS  Clinical Problems Assembly Programming Committee.

Key Learning Points

Patients with advanced COPD should also be considered for lung transplantation. We will have an episode on lung transplant coming up soon!

References

  1. Criner GJ, Sternberg AL. A Clinician’s Guide to the Use of Lung Volume Reduction Surgery. Proc Am Thorac Soc. 2008;5(4):461-467. doi:10.1513/pats.200709-151ET
  2. A Randomized Trial Comparing Lung-Volume–Reduction Surgery with Medical Therapy for Severe Emphysema. New England Journal of Medicine. 2003;348(21):2059-2073. doi:10.1056/NEJMoa030287
  3. Valipour A, Slebos DJ, Herth F, et al. Endobronchial Valve Therapy in Patients with Homogeneous Emphysema. Results from the IMPACT Study. Am J Respir Crit Care Med. 2016;194(9):1073-1082. doi:10.1164/rccm.201607-1383OC
  4. Sciurba FC, Ernst A, Herth FJF, et al. A Randomized Study of Endobronchial Valves for Advanced Emphysema. New England Journal of Medicine. 2010;363(13):1233-1244. doi:10.1056/NEJMoa0900928
  5. Klooster K, Slebos DJ. Endobronchial Valves for the Treatment of Advanced Emphysema. Chest. 2021;159(5):1833-1842. doi:10.1016/j.chest.2020.12.007
  6. Choi M, Lee WS, Lee M, et al. Effectiveness of bronchoscopic lung volume reduction using unilateral endobronchial valve: a systematic review and meta-analysis. Int J Chron Obstruct Pulmon Dis. 2015;10:703-710. doi:10.2147/COPD.S75314

Radiology Rounds – 5/3/22

This week on #RadiologyRounds we continue our series on COPD. Make sure to listen to all our episodes made in collaboration with the ATS Clinical Problems Assembly.

The CT shows moderate to severe centrilobular emphysema. These different patterns of emphysematous changes on CT can be related to the underlying driver of the disease and to symptom and disease severity.

In terms of follow-up, would you test this patient for alpha 1 anti-trypsin deficiency?

Current GOLD guidelines recommend that everyone with COPD, regardless of age or ethnicity should be tested for alpha 1 anti-trypsin deficiency.

15. COPD Exacerbations

Today we have episode two in our Pulm PEEPs and ATS Clinical Problems Assembly collaborative series on COPD. We are joined by Dr. Brad Drummond and Dr. Allison Lambert to discuss COPD exacerbations. Make sure to check out episode 1 in the series and stay tuned for more great content.

Meet Our Guests

Brad Drummond is an Associate Professor of Medicine at UNC School of Medicine. He is also the Associate Division Chief of Outpatient Services, the Co-Medical Director of the Pulmonary Specialty Clinics at UNC, and the Director of the Obstructive Lung Diseases Clinical and Translational Research Center. He is also the incoming Assembly Chair for the ATS CP Assembly.

Allison Lambert is a Pulmonary and Critical Care physician at Providence Medical Group, where she is also the Director of the Adult Cystic Fibrosis Program and co-leads the Therapeutic Development Network. Her expertise spans CF, non-CF bronchiectasis as well as COPD. Allison is also a committee member in the ATS Clinical Problems Assembly

Key Learning Points

References

  1. Wedzicha JA, Miravitlles M, Hurst JR, et al. Management of COPD exacerbations: a European Respiratory Society/American Thoracic Society guideline. European Respiratory Journal. 2017;49(3). doi:10.1183/13993003.00791-2016
  2. Lindenauer PK, Dharmarajan K, Qin L, Lin Z, Gershon AS, Krumholz HM. Risk Trajectories of Readmission and Death in the First Year after Hospitalization for Chronic Obstructive Pulmonary Disease. Am J Respir Crit Care Med. 2018;197(8):1009-1017. doi:10.1164/rccm.201709-1852OC
  3. Leuppi JD, Schuetz P, Bingisser R, et al. Short-term vs Conventional Glucocorticoid Therapy in Acute Exacerbations of Chronic Obstructive Pulmonary Disease: The REDUCE Randomized Clinical Trial. JAMA. 2013;309(21):2223-2231. doi:10.1001/jama.2013.5023
  4. Johns Hopkins University. Roflumilast or Azithromycin to Prevent COPD Exacerbations (RELIANCE). clinicaltrials.gov; 2022. Accessed April 24, 2022. https://clinicaltrials.gov/ct2/show/NCT04069312
  5. Barnes PJ. Chronic Obstructive Pulmonary Disease. New England Journal of Medicine. 2000;343(4):269-280. doi:10.1056/NEJM200007273430407
  6. Celli BR, Wedzicha JA. Update on Clinical Aspects of Chronic Obstructive Pulmonary Disease. New England Journal of Medicine. 2019;381(13):1257-1266. doi:10.1056/NEJMra1900500
  7. Singh D, Agusti A, Anzueto A, et al. Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Lung Disease: the GOLD science committee report 2019. Eur Respir J. 2019;53(5):1900164. doi:10.1183/13993003.00164-2019

13. COPD Classification and Practical Management Strategies

The Pulm PEEPs are extremely excited today to be launching our series on Chronic Obstructive Pulmonary Disease in partnership with the ATS Clinical Problems Assembly. We are joined by Dr. Bob Wise and Dr. Wassim Labaki to discuss the classification and initial workup of COPD, and management strategies from inhalers to pulmonary rehabilitation. Make sure to listen today and in the coming weeks for the rest of our COPD discussion.

Meet Our Guests

Dr. Bob Wise is a Professor of Medicine at Johns Hopkins School of Medicine and has served as the Medical Director of the Pulmonary Function Lab at the Johns Hopkins Asthma and Allergy Center. Bob is a leader in the care of patients with obstructive lung disease and his research focus has been conducting multi-center clinical trials in airway disease and is also a master physiologist. Bob has been involved in various capacities with ATS throughout his tenure as well and received the ATS CP Assembly Sreedhar Nair Lifetime Achievement Award in COPD.

Dr. Wassim Labaki is an Assistant Professor of Medicine in the Division of Pulmonary and Critical Care Medicine as well as the Medical Director of the Lung Volume Reduction Surgery Program at the University of Michigan. Wassim was the recipient of the Early Career Investigator Award in COPD from ATS in 2019 and currently is on the Program Committee of the ATS Clinical Problems Assembly.

Key Learning Points

Modified Medical Research Council (mMRC) Dyspnea Scale

mMRC Grade 0 = Only breathless with strenuous exercise

mMRC Grade 1 = Short of breath when hurrying on level ground, or walking up a slight hill

mMRC Grade 2 = Walking slower than people of the same age due to dyspnea, or stopping due to dyspnea when walking at my own pace on level ground

mMRC Grade 3 = Stopping for breath after walking 100 meters / a few minutes on level ground

mMRC Grade 4 = Too breathless to leave the house or breathless with getting dressed / undressed

Image source: Global Initiative for Chronic Obstructive Lung Disease https://goldcopd.org/

References and links for further reading

  1. Clinicians. Global Initiative for Chronic Obstructive Lung Disease – GOLD. Accessed April 11, 2022. https://goldcopd.org/clinicians/
  2. Miami CF 3300 P de LB. COPD Foundation | Take Action Today. Breathe Better Tomorrow. Accessed April 11, 2022. https://www.copdfoundation.org
  3. Barnes PJ. Chronic Obstructive Pulmonary Disease. New England Journal of Medicine. 2000;343(4):269-280. doi:10.1056/NEJM200007273430407
  4. Celli BR, Wedzicha JA. Update on Clinical Aspects of Chronic Obstructive Pulmonary Disease. New England Journal of Medicine. 2019;381(13):1257-1266. doi:10.1056/NEJMra1900500
  5. Criner GJ, Martinez FJ, Aaron S, et al. Current Controversies in Chronic Obstructive Pulmonary Disease. A Report from the Global Initiative for Chronic Obstructive Lung Disease Scientific Committee. Ann Am Thorac Soc. 2019;16(1):29-39. doi:10.1513/AnnalsATS.201808-557PS
  6. Martinez FJ, Agusti A, Celli BR, et al. Treatment Trials in Young Patients with Chronic Obstructive Pulmonary Disease and Pre-Chronic Obstructive Pulmonary Disease Patients: Time to Move Forward. Am J Respir Crit Care Med. 2022;205(3):275-287. doi:10.1164/rccm.202107-1663SO
  7. Rodriguez-Roisin R, Rabe KF, Vestbo J, Vogelmeier C, Agustí A, all previous and current members of the Science Committee and the Board of Directors of GOLD (goldcopd.org/committees/). Global Initiative for Chronic Obstructive Lung Disease (GOLD) 20th Anniversary: a brief history of time. Eur Respir J. 2017;50(1):1700671. doi:10.1183/13993003.00671-2017
  8. Singh D, Agusti A, Anzueto A, et al. Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Lung Disease: the GOLD science committee report 2019. Eur Respir J. 2019;53(5):1900164. doi:10.1183/13993003.00164-2019

7. Top Consults: Severe Asthma Exacerbation

We are excited to bring you another episode in our Pulm PEEPs Top Consults series! Kristina Montemayor and David Furfaro, are joined by Sandy Zaeh to discuss the assessment and management of a patient with a severe asthma exacerbation. We’ll follow a consult patient from the emergency department to the ICU, and cover everything from the physiology of pulsus paradoxus in asthma to how to manage the ventilator in status asthmaticus. Listen today and please send any questions our way on Twitter @pulmPEEPS.

Meet Our Guests

Sandy Zaeh is an Instructor of Medicine and Pulmonary & Critical Care Medicine physician at Yale School of Medicine.

Key Learning Points

References and links for further reading

  1. Chung KF, Wenzel SE, Brozek JL, et al. International ERS/ATS guidelines on definition, evaluation and treatment of severe asthma. European Respiratory Journal. 2014;43(2):343-373. doi:10.1183/09031936.00202013
  2. Rodrigo GJ, Rodrigo C, Hall JB. Acute asthma in adults: a review. Chest. 2004;125(3):1081-1102. doi:10.1378/chest.125.3.1081
  3. Godwin HT, Fix ML, Baker O, Madsen T, Walls RM, Brown CA. Emergency Department Airway Management for Status Asthmaticus With Respiratory Failure. Respir Care. 2020;65(12):1904-1907. doi:10.4187/respcare.07723
  4. Althoff MD, Holguin F, Yang F, et al. Noninvasive Ventilation Use in Critically Ill Patients with Acute Asthma Exacerbations. Am J Respir Crit Care Med. 2020;202(11):1520-1530. doi:10.1164/rccm.201910-2021OC
  5. Brenner B, Corbridge T, Kazzi A. Intubation and Mechanical Ventilation of the Asthmatic Patient in Respiratory Failure. Proc Am Thorac Soc. 2009;6(4):371-379. doi:10.1513/pats.P09ST4
  6. Laher AE, Buchanan SK. Mechanically Ventilating the Severe Asthmatic. J Intensive Care Med. 2018;33(9):491-501. doi:10.1177/0885066617740079
  7. Leatherman J. Mechanical ventilation for severe asthma. Chest. 2015;147(6):1671-1680. doi:10.1378/chest.14-1733

5. A Case of Chronic, Productive Cough

The Pulm PEEPs are joined again by Natalie West to discuss a patient who presented with a chronic, productive cough. Listen in today as we work through our differential diagnosis, interpret basic pulmonary testing, and share our clinical reasoning along the way. We have some fantastic diagnostic and treatment teaching points, so once you’ve solved the case check out the takeaways and infographics below. Please let us know any additional insights you have on Twitter!

Patient Presentation

A 50-year-old woman, who is a never smoker, with a past medical history of recurrent pancreatitis presents to the pulmonary clinic with a chronic, productive cough. Her cough has been present for 3 years and has increased in frequency to now being present daily. In the last three months, the cough has also worsened and is productive of small amounts of yellow to green sputum. She has a history of chronic post-nasal drip and sinus infections, and uses intranasal steroids, but has not noted changes in these symptoms. There is no significant family history of pulmonary disease, and an exposure history review of symptoms is negative.

On physical exam, she was a thin woman who appeared her stated age and was breathing comfortably on room air. Her exam was notable for mild expiratory wheezing, primarily on auscultation of the right posterior lung field. She had no cyanosis, clubbing, evidence of volume overload, or abdominal tenderness.

Basic Spirometry Values
Chest X-ray

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below.

Differential Diagnosis of Chronic Cough

Three most common causes: upper airway cough syndrome, GERD, cough variant asthma

Additional etiologies to consider: chronic bronchitis, post-infectious after a respiratory tract infection, bronchiectasis, ACE inhibitors, lung cancer, eosinophilic bronchitis, interstitial lung disease


Imaging Pearl


Evaluating Bronchiectasis


Making a New Diagnosis of Cystic Fibrosis in an Adult

Sweat testing

Sweat testing should be done in CF accredited center. Inform patients that there are no needles involved. Pilocarpine and electrical stimulation are applied to the arm or leg to stimulate the sweat gland, and then sweat is collected on filter paper, a gauze, or a plastic coil. From there, the amount of chloride in the sweat is calculated

Results

< 30 normal

31 – 60 indeterminate

> 60 is positive and Cystic Fibrosis is likely

What do you do with an Indeterminate test?

Patients with milder phenotypes of Cystic Fibrosis can have a normal or indeterminate sweat chloride level, and 10% of adults diagnosed with CF have a normal sweat chloride. If the sweat chloride test is indeterminate or normal, but suspicion is high for CF, then genetic testing for the whole array of mutations should be performed

References and links for further reading

  1. Morice AH, Millqvist E, Bieksiene K, et al. ERS guidelines on the diagnosis and treatment of chronic cough in adults and children. European Respiratory Journal. 2020;55(1). doi:10.1183/13993003.01136-2019
  2. Barker AF. Bronchiectasis. New England Journal of Medicine. 2002;346(18):1383-1393. doi:10.1056/NEJMra012519
  3. Bronchiectasis: a case-based approach to investigation and management | European Respiratory Society. Accessed November 23, 2021. https://err.ersjournals.com/content/27/149/180016
  4. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. New England Journal of Medicine. 2005;352(19):1992-2001. doi:10.1056/NEJMra043184
  5. Shteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. The Lancet. 2021;397(10290):2195-2211. doi:10.1016/S0140-6736(20)32542-3
  6. Jain R. Diagnosing Cystic Fibrosis in Adults: Better Late Than Never. Annals ATS. 2018;15(10):1140-1141. doi:10.1513/AnnalsATS.201806-432ED

3. A Case of Worsening Episodic Dyspnea

The Pulm PEEPs are excited to bring our first mystery case! Kristina Montemayor and Dave Furfaro hear a fascinating case presentation from Pulm PEEPs senior editor Ansa Razzaq. Join us as we work through this case together to come to a diagnosis, and share our thought process along the way. Come back to these show notes afterward, or once you’ve solved the case yourself, for some key teaching pearls and representative images.

Patient Presentation

A 66-year-old woman with no smoking history and past medical history of previously well-controlled asthma is referred to pulmonary clinic after multiple recent episodes of dyspnea, wheezing, and coughing. The episodes have features consistent with asthma exacerbations; however, they are also associated with migratory infiltrates. She has been treated with multiple courses of antibiotics and steroids, and despite escalating therapy, the episodes are occurring more frequently and she was worsening overall exercise tolerance. Listen in to hear more and try to solve the case!

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below.

References and links for further reading

  1. Israel E, Reddel HK. Severe and Difficult-to-Treat Asthma in Adults. New England Journal of Medicine. 2017;377(10):965-976. doi:10.1056/NEJMra1608969
  2. Asthma NAE and PP Third Expert Panel on the Diagnosis and Management of. Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma. National Heart, Lung, and Blood Institute (US); 2007.
  3. Chung KF, Wenzel SE, Brozek JL, et al. International ERS/ATS guidelines on definition, evaluation and treatment of severe asthma. European Respiratory Journal. 2014;43(2):343-373. doi:10.1183/09031936.00202013
  4. Barker AF. Bronchiectasis. New England Journal of Medicine. 2002;346(18):1383-1393. doi:10.1056/NEJMra012519
  5. Chen T hsu, Hollingsworth H. Allergic Bronchopulmonary Aspergillosis. New England Journal of Medicine. 2008;359(6):e7. doi:10.1056/NEJMicm055764
  6. Agarwal R, Dhooria S, Singh Sehgal I, et al. A Randomized Trial of Itraconazole vs Prednisolone in Acute-Stage Allergic Bronchopulmonary Aspergillosis Complicating Asthma. Chest. 2018;153(3):656-664. doi:10.1016/j.chest.2018.01.005

2. Cystic Fibrosis Roundtable

The Pulm PEEPs (Kristina Montemayor and Dave Furfaro) host a panel of Cystic Fibrosis (CF) providers to discuss the current state of the disease, recent advances in cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, and the evolving faces and voices of Cystic Fibrosis.

Cystic Fibrosis is an autosomal recessive disorder caused by mutations in the CFTR gene that affects over 30,000 individuals in the United States and 70,000 people worldwide. Absence or dysfunction of the CFTR protein leads to abnormal secretion of mucus, sweat, and digestive fluids, which impacts the lungs, digestive tract, and reproductive system.

From the first formal publication on Cystic Fibrosis in 1938 by Dorothy Hansine Andersen, to the discovery of the delta F508 mutation and CFTR gene in 1988 -1989 by Lap-Chee Tsui, Francis Collins, and John R. Riordan, to the approval of the first CFTR modulator therapy, Ivacaftor, in 2012, our knowledge about Cystic FIbrosis has been advancing in leaps and bounds. As therapies have improved, they have dramatically impacted the lives of patients with Cystic Fibrosis. Join us today as we explore what this evolution in care has looked like from the perspective of Cystic Fibrosis providers, and hear about the new questions and challenges on the horizon.


Meet our guests

Emily DiMango is a Professor of Medicine at Columbia University Medical Center and the Director of the John Edsall-John Wood Asthma Center and the Gunnar Esiason Adult Cystic Fibrosis Program

Terri Laguna is an Associate Professor of Pediatrics at Northwestern Medicine / Feinberg School of Medicine and the Chief of Pulmonary and Sleep Medicine in the Department of Pediatrics

Patrick Sosnay is a Senior Medical Director at Vertex Pharmaceuticals and specializes in Cystic Fibrosis

Natalie West is an Assistant Professor of Medicine at Johns Hopkins Hospital and specializes in Cystic Fibrosis.


References and links for further reading

  1. Cystic Fibrosis Foundation
  2. Shteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. The Lancet. 2021;397(10290):2195-2211. doi:10.1016/S0140-6736(20)32542-3
  3. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. New England Journal of Medicine. 2005;352(19):1992-2001. doi:10.1056/NEJMra043184
  4. Davis PB. Cystic Fibrosis Since 1938. Am J Respir Crit Care Med. 2006;173(5):475-482. doi:10.1164/rccm.200505-840OE
  5. Barry PJ, Mall MA, Álvarez A, et al. Triple Therapy for Cystic Fibrosis Phe508del–Gating and –Residual Function Genotypes. New England Journal of Medicine. 2021;385(9):815-825. doi:10.1056/NEJMoa2100665
  6. Middleton PG, Mall MA, Dřevínek P, et al. Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. New England Journal of Medicine. Published online October 31, 2019. doi:10.1056/NEJMoa1908639