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.

[1]  A. Nicholson,et al.  Clinical significance of respiratory bronchiolitis on open lung biopsy and its relationship to smoking related interstitial lung disease , 1999, Thorax.

[2]  Standardization of spirometry--1987 update. Statement of the American Thoracic Society. , 1987, The American review of respiratory disease.

[3]  D Curran-Everett,et al.  Multiple comparisons: philosophies and illustrations. , 2000, American journal of physiology. Regulatory, integrative and comparative physiology.

[4]  F. Lake,et al.  Respiratory bronchiolitis associated interstitial lung disease (RB‐ILD) presenting with haemoptysis , 2000, Respirology.

[5]  D. Niewoehner,et al.  Pathologic changes in the peripheral airways of young cigarette smokers. , 1974, The New England journal of medicine.

[6]  J. Tooze,et al.  Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. , 2001, American journal of respiratory and critical care medicine.

[7]  J. Myers,et al.  Respiratory bronchiolitis causing interstitial lung disease. A clinicopathologic study of six cases. , 1987, The American review of respiratory disease.

[8]  H Weill,et al.  Ethnic differences in lung function: evidence for proportional differences. , 1974, International journal of epidemiology.

[9]  E. Žuškin,et al.  Cigarette Smoking: Objective Evidence for Lung Damage in Teen-Agers , 1971, Science.

[10]  D A Lynch,et al.  Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process? , 1999, AJR. American journal of roentgenology.

[11]  Elisa T. Lee,et al.  Statistical Methods for Survival Data Analysis , 1994, IEEE Transactions on Reliability.

[12]  T. Colby,et al.  Respiratory bronchiolitis-associated interstitial lung disease and its relationship to desquamative interstitial pneumonia. , 1989, Mayo Clinic proceedings.

[13]  R. Forster,et al.  A standardized breath holding technique for the clinical measurement of the diffusing capacity of the lung for carbon monoxide. , 1957, The Journal of clinical investigation.

[14]  M. Schwarz,et al.  Idiopathic pulmonary fibrosis. Pretreatment bronchoalveolar lavage cellular constituents and their relationships with lung histopathology and clinical response to therapy. , 1987, The American review of respiratory disease.

[15]  A. Katzenstein,et al.  Respiratory Bronchiolitis: A Clinicopathologic Study in Current Smokers, Ex-Smokers, and Never-Smokers , 2002, The American journal of surgical pathology.

[16]  Goldman Hi,et al.  Respiratory function tests; normal values at median altitudes and the prediction of normal results. , 1959 .

[17]  M. Schwarz,et al.  A clinical, radiographic, and physiologic scoring system for the longitudinal assessment of patients with idiopathic pulmonary fibrosis. , 1986, The American review of respiratory disease.

[18]  A. Morris Clinical pulmonary function testing : a manual of uniform laboratory procedures , 1984 .

[19]  D. Lynch,et al.  Respiratory Bronchiolitis-Associated Interstitial Lung Disease: Radiologic Features with Clinical and Pathologic Correlation , 2002, Journal of computer assisted tomography.

[20]  Paul J. Friedman,et al.  American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors , 2002, American journal of respiratory and critical care medicine.

[21]  A. Nicholson,et al.  Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease , 2001, Seminars in respiratory and critical care medicine.

[22]  P. Dinakara,et al.  The effect of anemia on pulmonary diffusing capacity with derivation of a correction equation. , 2015, The American review of respiratory disease.