Respiratory bronchiolitis-interstitial lung disease
Citation, DOI & article data
Respiratory bronchiolitis interstitial lung disease (RB-ILD) is a smoking related interstitial lung disease closely related to respiratory bronchiolitis, but demonstrating more severe histological, imaging and clinical findings.
In all cases, respiratory bronchiolitis-interstitial lung disease is typically associated with heavy tobacco smoking (usually of 30 pack-years or more) and is often seen in young middle-aged patients (30-40 years of age).
Presentation tends to be with progressive shortness of breath and chronic cough 4. Inspiratory crackles are heard in up to half of patients. Clubbing is rare 1.
On lung biopsy of respiratory bronchiolitis-interstitial lung disease, pigment-laden macrophages are found within alveoli, and peribronchial inflammation noted. Unlike simple respiratory bronchiolitis, fibrosis extends beyond the tissues immediately adjacent to respiratory bronchioles and extends into alveolar septae.
Histologically respiratory bronchiolitis-interstitial lung disease is very similar to desquamative interstitial pneumonia (DIP) and many authors feel that the two conditions are closely related, or even variants of the same condition 3. It has even been suggested that DIP should be abolished altogether, and simply thought of as end-stage/severe RB-ILD 5.
- ground glass opacities: may have a slight upper zone predilection 9
- poorly defined centrilobular nodules
- no particular distribution with both upper and lower zones potentially affected
- if advanced, fibrosis may be evident typically affecting the subpleural regions and more so in the lower lung zones
- patchy areas of hypoattenuation (~40%) with a lower lung predominance 10
- other changes related to smoking
Treatment and prognosis
Usually, no treatment is required for respiratory bronchiolitis-interstitial lung disease other than giving up smoking.
History and etymology
It was first described in 1974 by the American pulmonologist Dennis E Niewoehner (1940-2020) 15 and colleagues 11,13.
With typical HRCT chest appearances consider:
- non-specific interstitial pneumonitis (NSIP)
- acute or subacute hypersensitivity pneumonitis (HP)
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- 2. Miller W & Shah R. Isolated Diffuse Ground-Glass Opacity in Thoracic CT: Causes and Clinical Presentations. AJR Am J Roentgenol. 2005;184(2):613-22. doi:10.2214/ajr.184.2.01840613 - Pubmed
- 3. Heyneman L, Ward S, Lynch D, Remy-Jardin M, Johkoh T, Müller N. Respiratory Bronchiolitis, Respiratory Bronchiolitis-Associated Interstitial Lung Disease, and Desquamative Interstitial Pneumonia: Different Entities or Part of the Spectrum of the Same Disease Process? AJR Am J Roentgenol. 1999;173(6):1617-22. doi:10.2214/ajr.173.6.10584810 - Pubmed
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- 7. Moon J, du Bois R, Colby T, Hansell D, Nicholson A. Clinical Significance of Respiratory Bronchiolitis on Open Lung Biopsy and Its Relationship to Smoking Related Interstitial Lung Disease. Thorax. 1999;54(11):1009-14. doi:10.1136/thx.54.11.1009 - Pubmed
- 8. Wells A, Nicholson A, Hansell D, du Bois R. Respiratory Bronchiolitis-Associated Interstitial Lung Disease. Semin Respir Crit Care Med. 2003;24(5):585-94. doi:10.1055/s-2004-815606 - Pubmed
- 9. Mavridou D & Laws D. Respiratory Bronchiolitis Associated Interstitial Lung Disease (RB-ILD): A Case of an Acute Presentation. Thorax. 2004;59(10):910-1. doi:10.1136/thx.2003.011080 - Pubmed
- 10. Park J, Brown K, Tuder R, Hale V, King T, Lynch D. Respiratory Bronchiolitis-Associated Interstitial Lung Disease: Radiologic Features with Clinical and Pathologic Correlation. J Comput Assist Tomogr. 2002;26(1):13-20. doi:10.1097/00004728-200201000-00003 - Pubmed
- 11. Niewoehner D, Kleinerman J, Rice D. Pathologic Changes in the Peripheral Airways of Young Cigarette Smokers. N Engl J Med. 1974;291(15):755-8. doi:10.1056/NEJM197410102911503 - Pubmed
- 12. Woo O, Yong H, Oh Y, Lee S, Kim H, Kang E. Respiratory Bronchiolitis-Associated Interstitial Lung Disease in a Nonsmoker: Radiologic and Pathologic Findings. AJR Am J Roentgenol. 2007;188(5):W412-4. doi:10.2214/AJR.05.0835 - Pubmed
- 13. Krishnan J, Voelker H, Connett J et al. Effect of Daily Azithromycin Therapy and Adherence on Readmission Risk in COPD. Eur Respir J. 2019;53(3):1801377. doi:10.1183/13993003.01377-2018 - Pubmed
- 14. Sieminska A & Kuziemski K. Respiratory Bronchiolitis-Interstitial Lung Disease. Orphanet J Rare Dis. 2014;9(1):106. doi:10.1186/s13023-014-0106-8 - Pubmed
- 15. Dennis E. Niewoehner obituary. Star Tribune. https://www.startribune.com/obituaries/detail/0000367137/ [accessed 3 June 2022].