R2 Talk
Interstitial Lung Disease
Dr. Paul Mester
Mentor: Dr. Douglas Hornick, Departement of Pulmonary and Critical Care Medicine
Interstitial Lung Disease
History: look for history of cigarette smoking, environmental exposures, history of autoimmune disease
Symptoms: cough, dyspnea on exertion; may also have associated autoimmune symptoms (joint pains/swelling, Raynaud’s muscle weakness rash, GERD etc)
Physical exam: crackles, “Velcro” rales, finger clubbing (also look for extrapulmonary disease manifestations e.g. synovitis, Bouchard’s nodes, skin thickening/tightening etc)
Work-up: high-resolution CT and PFTs are most important. Also consider chest x-ray and autoimmune/infectious labs (ESR/CRP, ANCAs, RF/CCP, ANA/dsDNA, Scl-70/centromere, myositis panel/CK/aldolase, HIV etc)
Treatment: referral to appropriate sub-specialists (e.g., pulmonary, rheumatology) for further evaluation. Depending upon etiology treatment may include glucocorticoids, pirfenidone, nintedanib and/or immunomodulatory drugs (methotrexate, MMF, rituximab etc).
Pneumococcal vaccination and flu vaccinations are indicated
Smoking cessation is recommended
Sources
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Lederer DJ, Martinez FJ. Idiopathic Pulmonary Fibrosis. N Engl J Med. 2018;378(19):1811-1823. doi:10.1056/NEJMra1705751
Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076. Published 2018 Dec 21. doi:10.1183/16000617.0076-2018
Antoniou KM, Margaritopoulos GA, Tomassetti S, Bonella F, Costabel U, Poletti V. Interstitial lung disease. Eur Respir Rev. 2014;23(131):40-54. doi:10.1183/09059180.00009113
Watanabe K. Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics. Curr Respir Med Rev. 2013;9(4):299-237. doi:10.2174/1573398X0904140129125307
Panchabhai TS, Farver C, Highland KB. Lymphocytic Interstitial Pneumonia. Clin Chest Med. 2016;37(3):463-474. doi:10.1016/j.ccm.2016.04.009
Mrad A, Huda N. Acute Interstitial Pneumonia. In: StatPearls. Treasure Island (FL): StatPearls Publishing; August 11, 2020.
Chandra D, Maini R, Hershberger DM. Cryptogenic Organizing Pneumonia. In: StatPearls. Treasure Island (FL): StatPearls Publishing; September 18, 2020.
Margaritopoulos GA, Harari S, Caminati A, Antoniou KM. Smoking-related idiopathic interstitial pneumonia: A review. Respirology. 2016;21(1):57-64. doi:10.1111/resp.12576
Chakraborty RK, Basit H, Sharma S. Desquamative Interstitial Pneumonia. In: StatPearls. Treasure Island (FL): StatPearls Publishing; August 12, 2020.
