Respiratory bronchiolitis-interstitial lung disease: long-term outcome

Chest. 2007 Mar;131(3):664-671. doi: 10.1378/chest.06-1885.


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.

MeSH terms

  • Administration, Inhalation
  • Adrenal Cortex Hormones / administration & dosage
  • Adult
  • Aged
  • Bronchiolitis / diagnosis*
  • Bronchiolitis / mortality
  • Bronchiolitis / physiopathology
  • Bronchiolitis / therapy
  • Cohort Studies
  • Combined Modality Therapy
  • Disease Progression
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Forced Expiratory Volume / physiology
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / mortality
  • Lung Diseases, Interstitial / physiopathology
  • Lung Diseases, Interstitial / therapy
  • Male
  • Middle Aged
  • Oxygen / blood
  • Pulmonary Diffusing Capacity / drug effects
  • Pulmonary Diffusing Capacity / physiology
  • Pulmonary Gas Exchange / drug effects
  • Pulmonary Gas Exchange / physiology
  • Rare Diseases / diagnosis*
  • Rare Diseases / mortality
  • Rare Diseases / physiopathology
  • Rare Diseases / therapy
  • Retrospective Studies
  • Smoking / adverse effects
  • Smoking Cessation
  • Survival Rate
  • Vital Capacity / drug effects
  • Vital Capacity / physiology


  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Oxygen