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Published online before print January 7, 2009, 10.1183/09031936.00091608
Eur Respir J 2009; 33:1053-1061
Copyright ©ERS Journals Ltd 2009
doi: 10.1183/09031936.00091608

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Characterisation of severe obliterative bronchiolitis in rheumatoid arthritis

G. Devouassoux1,7, V. Cottin2,7, H. Lioté3, E. Marchand4, I. Frachon5, A. Schuller1, F. Béjui-Thivolet6, J-F. Cordier2 and the Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P)

1 Hospices civils de Lyon, Centre Hospitalier Lyon Sud, Service de Pneumologie, Pierre-Bénite, 2 Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie, Centre de Référence des maladies pulmonaires rares de l'adulte, Université de Lyon, Université Lyon I, 6 Hospices civils de Lyon, Hôpital Louis Pradel, Service de Cytologie et Anatomie Pathologique, Lyon, 3 AP-HP, Hôpital Tenon, Service de Pneumologie et Réanimation, Centre de Compétence des maladies pulmonaires rares de l'adulte, Paris, 4 Cliniques Universitaires UCL de Mont-Godinne, Yvoir, 5 Service de Médecine Interne et Pneumologie, Hôpital de la Cavale Blanche, Brest, France, 7 These authors contributed equally to this work

CORRESPONDENCE: J-F. Cordier, Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires, Hôpital Louis Pradel, 69677 Lyon (Bron), France. Fax: 33 472357653. E-mail: germop{at}univ-lyon1.fr

Keywords: Airflow obstruction, bronchiectasis, emphysema, obliterative bronchiolitis, rheumatoid arthritis, small airways disease

Received: June 16, 2008
Accepted November 19, 2008

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.






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