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Respiratory bronchiolitis-interstitial lung disease: long-term outcome
Joshua Portnoy1, Kristen L Veraldi2, Marvin I Schwarz2
1National Jewish Medical and Research Center, Denver, CO.
Chest
|March 16, 2007
Summary
Respiratory bronchiolitis (RB)-interstitial lung disease (ILD) patients often survive long-term, but symptomatic and physiologic improvement is rare. Smoking cessation and immunosuppressive therapies show limited clinical benefit in this condition.
Area of Science:
- Pulmonology
- Interstitial Lung Diseases
- Clinical Outcomes Research
Background:
- Respiratory bronchiolitis (RB)-interstitial lung disease (ILD) is a known condition, but its natural history and patient outcomes require further systematic evaluation.
- Previous reports suggest RB-ILD is nonprogressive and improves with smoking cessation and anti-inflammatory treatment.
Purpose of the Study:
- To determine the long-term outcome of RB-ILD patients.
- To evaluate the impact of smoking cessation and corticosteroid therapy on RB-ILD outcomes.
Main Methods:
- A cohort of 32 RB-ILD patients confirmed by surgical lung biopsy was prospectively enrolled.
- Data on symptoms, lung function, treatments, and outcomes were collected and analyzed using Kaplan-Meier survival analysis.
Main Results:
- At least 75% of RB-ILD patients survived over 7 years post-diagnosis.
- Clinical improvement was observed in only 28% of patients, and physiologic improvement in 10.5%.
- Physiologic improvement was associated with smoking cessation; corticosteroids had minimal impact on symptoms or physiology.
Conclusions:
- Prolonged survival is common in RB-ILD.
- Symptomatic and physiologic improvement is infrequent in RB-ILD patients.
- Neither smoking cessation nor immunosuppressive therapy consistently leads to clinically significant benefits.
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