Respiratory bronchiolitis-interstitial lung disease: long-term outcome.
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.
Portnoy
Joshua
J
National Jewish Medical and Research Center, Denver, CO.
Veraldi
Kristen L
KL
University of Colorado Health Sciences Center, Denver, CO.
Schwarz
Marvin I
MI
University of Colorado Health Sciences Center, Denver, CO.
Cool
Carlyne D
CD
National Jewish Medical and Research Center, Denver, CO.
Curran-Everett
Douglas
D
National Jewish Medical and Research Center, Denver, CO.
Cherniack
Reuben M
RM
National Jewish Medical and Research Center, Denver, CO.
King
Talmadge E
TE
University of California at San Francisco, San Francisco, CA.
Brown
Kevin K
KK
National Jewish Medical and Research Center, Denver, CO. Electronic address: brownk@njc.org.
D000280:Administration, Inhalation; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D000368:Aged; D001988:Bronchiolitis / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D015331:Cohort Studies; D003131:Combined Modality Therapy; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010100:Oxygen / Q000097:blood; D011653:Pulmonary Diffusing Capacity / Q000187:drug effects / Q000502:physiology; D011659:Pulmonary Gas Exchange / Q000187:drug effects / Q000502:physiology; D035583:Rare Diseases / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D012189:Retrospective Studies; D012907:Smoking / Q000009:adverse effects; D016540:Smoking Cessation; D015996:Survival Rate; D014797:Vital Capacity / Q000187:drug effects / Q000502:physiology
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D010100:Oxygen