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Respiratory Bronchiolitis-Interstitial Lung Disease^*

Long-term Outcome

Joshua Portnoy, MD; Kristen L. Veraldi, MD, PhD; Marvin I. Schwarz, MD, FCCP; Carlyne D. Cool, MD; Douglas Curran-Everett, PhD; Reuben M. Cherniack, MD; Talmadge E. King, Jr, MD, FCCP and Kevin K. Brown, MD, FCCP

^* From the National Jewish Medical and Research Center (Drs. Portnoy, Cool, Curran-Everett, Cherniack, and Brown), Denver, CO; University of Colorado Health Sciences Center (Drs. Schwarz and Veraldi), Denver, CO; and University of California at San Francisco (Dr. King), San Francisco, CA. These authors contributed equally to this article.

Correspondence to: Kevin K. Brown, MD, FCCP, National Jewish Medical and Research Center, 1400 Jackson St, Denver, CO 80206; e-mail: brownk{at}njc.org

Abstract

Background: The clinical and physiologic features of respiratory bronchiolitis (RB)-interstitial lung disease (ILD) have been previously described; however, the natural history and outcome have not been systematically evaluated. The majority of published reports consider RB-ILD to be a nonprogressive ILD that clinically improves with smoking cessation and antiinflammatory treatment. In this study, we sought to determine the outcome of RB-ILD patients with and without smoking cessation and with and without corticosteroid therapy.

Methods: Thirty-two RB-ILD cases confirmed by surgical lung biopsy were identified from a prospectively enrolled cohort of subjects with ILD. Initial and follow-up data on symptoms, physiology, treatment, and outcome were collected and analyzed.

Results: Kaplan-Meier analysis revealed that at least 75% of RB-ILD patients survived > 7 years after diagnosis. Clinical improvement occurred in only 28% of cases, and physiologic improvement occurred in 10.5% of cases. One patient died of progressive ILD, and two patients died of non-small cell lung cancer. While physiologic improvement was limited to those who had ceased smoking, corticosteroids and/or other immunosuppressive therapy had little effect on symptoms or physiology.

Conclusions: This study shows that prolonged survival is common in RB-ILD. However, symptomatic and physiologic improvement occurs in only a minority of patients, and neither smoking cessation nor immunosuppressive therapy is regularly associated with clinically significant benefit.

Key Words: idiopathic interstitial pneumonia • interstitial lung disease • respiratory bronchiolitis • respiratory bronchiolitis-interstitial lung disease • smoking • survival

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