Respiratory bronchiolitis-interstitial lung disease
Citation, DOI, disclosures and article data
At the time the article was created Frank Gaillard had no recorded disclosures.
View Frank Gaillard's current disclosuresAt the time the article was last revised Daniel J Bell had no financial relationships to ineligible companies to disclose.
View Daniel J Bell's current disclosures- Respiratory bronchiolitis ILD (RB-ILD)
- RBILD (interstitial lung disease)
- Respiratory bronchiolitis interstitial lung disease
- RB-ILD (interstitial lung disease)
- Respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)
- Respiratory bronchiolitis-interstitial lung disease (RB-ILD)
Respiratory bronchiolitis interstitial lung disease (RB-ILD) is a clinical diagnosis. The CT features are indistinguishable from respiratory bronchiolitis (RB) and should be reported as such.
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Epidemiology
In almost all cases, respiratory bronchiolitis-interstitial lung disease is associated with heavy tobacco smoking (usually 30 pack-years or more) and is often seen in 30-40 years age group.
Clinical presentation
Chronic cough and progressive dyspnea 4 differentiate RB-ILD from RB. Inspiratory crackles are heard in up to half of patients. Clubbing is rare 1. RB-ILD is a clinical diagnosis.
Pathology
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.
Radiographic features
CT
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
bronchial wall thickening: can be central +/- peripheral
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.
Differential diagnosis
With typical HRCT chest appearances consider:
-
non-specific interstitial pneumonitis (NSIP)
changes of fibrosis: lung volume loss, reticular opacities, and traction bronchiectasis
ground glass opacities have a random distribution
non-fibrotic or fibrotic hypersensitivity pneumonitis (HP)
See also
References
- 1. Attili A, Kazerooni E, Gross B, Flaherty K, Myers J, Martinez F. Smoking-Related Interstitial Lung Disease: Radiologic-Clinical-Pathologic Correlation. Radiographics. 2008;28(5):1383-96; discussion 1396. doi:10.1148/rg.285075223 - Pubmed
- 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
- 4. David P. Naidich, Nestor L. Müller, W. Richard Webb. Computed Tomography and Magnetic Resonance of the Thorax. (2007) ISBN: 9780781757652 - Google Books
- 5. Katzenstein A & Myers J. Idiopathic Pulmonary Fibrosis: Clinical Relevance of Pathologic Classification. Am J Respir Crit Care Med. 1998;157(4 Pt 1):1301-15. doi:10.1164/ajrccm.157.4.9707039 - Pubmed
- 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. doi:10.1183/09031936.00015506 - Pubmed
- 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].
Incoming Links
- HRCT chest (protocol)
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- Tobacco use
- Small airways disease
- Desquamative interstitial pneumonia
- Smoking-related interstitial lung diseases
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- Idiopathic interstitial pneumonias (an approach on HRCT chest)
- Respiratory bronchiolitis
- Centrilobular lung nodules
- Inflammatory bronchiolitis
- Interstitial lung disease
- Pulmonary opacification
- Bronchiolitis
- Vaping-associated lung disease
- Medical abbreviations and acronyms (R)
- Bronchial wall thickening
- Idiopathic interstitial pneumonias
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