Respiratory bronchiolitis-interstitial lung disease
Citation, DOI and 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 (fl. 2019) 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 WT, Shah RM. Isolated diffuse ground-glass opacity in thoracic CT: causes and clinical presentations. AJR Am J Roentgenol. 2005;184 (2): 613-22. AJR Am J Roentgenol (full text) - Pubmed citation
- 3. Heyneman LE, Ward S, Lynch DA et-al. 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. AJR Am J Roentgenol (abstract) - Pubmed citation
- 4. Naidich DP, Srichai MB, Krinsky GA. Computed tomography and magnetic resonance of the thorax. Lippincott Williams & Wilkins. (2007) ISBN:0781757657. Read it at Google Books - Find it at Amazon
- 5. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am. J. Respir. Crit. Care Med. 1998;157 (4 Pt 1): 1301-15. Am. J. Respir. Crit. Care Med. (link) - Pubmed citation
- 6. Nakanishi M, Demura Y, Mizuno S et-al. Changes in HRCT findings in patients with respiratory bronchiolitis-associated interstitial lung disease after smoking cessation. Eur. Respir. J. 2007;29 (3): 453-61. Eur. Respir. J. (full text) - doi:10.1183/09031936.00015506 - Pubmed citation
- 7. Moon J, du Bois RM, Colby TV et-al. Clinical significance of respiratory bronchiolitis on open lung biopsy and its relationship to smoking related interstitial lung disease. Thorax. 1999;54 (11): 1009-14. Thorax (full text) - doi:10.1136/thx.54.11.1009 - Free text at pubmed - Pubmed citation
- 8. Wells AU, Nicholson AG, Hansell DM et-al. Respiratory bronchiolitis-associated interstitial lung disease. Semin Respir Crit Care Med. 2003;24 (5): 585-94. doi:10.1055/s-2004-815606 - Pubmed citation
- 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 - Free text at pubmed - Pubmed citation
- 10. Park JS, Brown KK, Tuder RM et-al. Respiratory bronchiolitis-associated interstitial lung disease: radiologic features with clinical and pathologic correlation. J Comput Assist Tomogr. 2002;26 (1): 13-20. Pubmed citation
- 11. Niewoehner DE, Kleinerman J, Rice DB. Pathologic changes in the peripheral airways of young cigarette smokers. N. Engl. J. Med. 1974;291 (15): 755-8. doi:10.1056/NEJM197410102911503 - Pubmed citation
- 12. Woo OH, Yong HS, Oh YW et-al. 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 citation
- 13. Krishnan JK, Voelker H, Connett JE, Niewoehner DE, Albert RK, Scanlon PD, Criner GJ, Dransfield MT, Han MK, Martinez FJ. Effect of daily azithromycin therapy and adherence on readmission risk in COPD. (2019) The European respiratory journal. doi:10.1183/13993003.01377-2018 - Pubmed
- 14. Sieminska A, Kuziemski K. Respiratory bronchiolitis-interstitial lung disease. (2014) Orphanet journal of rare diseases. 9: 106. doi:10.1186/s13023-014-0106-8 - Pubmed