PT  - JOURNAL ARTICLE
AU  - G. Devouassoux
AU  - V. Cottin
AU  - H. Lioté
AU  - E. Marchand
AU  - I. Frachon
AU  - A. Schuller
AU  - F. Béjui-Thivolet
AU  - J-F. Cordier
AU  - the Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P)
TI  - Characterisation of severe obliterative bronchiolitis in rheumatoid arthritis
AID  - 10.1183/09031936.00091608
DP  - 2009 May 01
TA  - European Respiratory Journal
PG  - 1053--1061
VI  - 33
IP  - 5
4099  - http://erj.ersjournals.com/content/33/5/1053.short
4100  - http://erj.ersjournals.com/content/33/5/1053.full
SO  - Eur Respir J2009 May 01; 33
AB  - The characteristics of patients with rheumatoid arthritis (RA) who develop obliterative bronchiolitis characterised by severe airflow obstruction have been hitherto poorly investigated. A retrospective study of 25 patients with RA and functional evidence of obliterative bronchiolitis (forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) <50% and/or residual volume (RV)/total lung capacity (TLC) >140% predicted) was conducted. Patients (mean±sd age 64±11 yrs) included 17 never-smokers and eight ex-smokers (10.5±5.4 pack-yrs). The diagnosis of RA preceded respiratory symptoms in 88% of cases. Dyspnoea on exertion was present in all patients and bronchorrhea in 44%. High-resolution computed tomography findings included: bronchial wall thickening (96%), bronchiectasis (40%), mosaic pattern (40%), centrilobular emphysema (56%), and reticular and/or ground-glass opacities (32%). Pulmonary function tests showed: FEV1 41±12% pred, FEV1/FVC 49±14%, FVC 70±20% pred, RV 148±68% pred and RV/TLC 142±34% pred. Lung biopsy, available in nine patients, demonstrated constrictive, follicular and mixed bronchiolitis. Patients were followed for 48.2±49 months. Treatment was poorly effective. Chronic respiratory failure occurred in 40% of patients, and four patients died. Obliterative bronchiolitis associated with rheumatoid arthritis is a severe and under-recognised condition leading to respiratory failure and death in a high proportion of patients.