Tag Archives: Obstructive Lung Disease

Obstructive Lung Disease

3. A Case of Worsening Episodic Dyspnea

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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!

Representative Imaging

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.

Images from 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.
Image from 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.

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

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2. Cystic Fibrosis Roundtable

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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

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