Obliterative bronchiolitis associated with rheumatoid arthritis: analysis of a single-center case series

• Main Authors: Erica Lin, Andrew H. Limper, Teng Moua
• Format: Article
• Language: English
• Published: BMC 2018-06-01
• Series: BMC Pulmonary Medicine
• Subjects: Rheumatoid arthritis; Obliterative bronchiolitis; Small airways disease
• Online Access: http://link.springer.com/article/10.1186/s12890-018-0673-x

Abstract Background Rheumatoid arthritis (RA) is a systemic autoimmune condition characterized by erosive inflammation of the joints. One rare pulmonary manifestation is obliterative bronchiolitis (OB), a small airways disease characterized by the destruction of bronchiolar epithelium and airflow obstruction. Methods We retrospectively reviewed the clinical data of patients with rheumatoid arthritis-associated obliterative bronchiolitis (RA-OB) from 01/01/2000 to 12/31/2015. Presenting clinical features, longitudinal pulmonary function testing, radiologic findings, and independent predictors of all-cause mortality were assessed. Results Forty one patients fulfilled criteria for diagnosis of RA-OB. There was notable female predominance (92.7%) with a mean age of 57 ± 15 years. Dyspnea was the most common presenting clinical symptom. Median FEV1 was 40% (IQR 31–52.5) at presentation, with a mean decline of − 1.5% over a follow-up period of thirty-three months. Associated radiologic findings included mosaic attenuation and pulmonary nodules. A majority of patients (78%) received directed therapy including long-acting inhalers, systemic corticosteroids or other immunosuppressive agents, and macrolide antibiotics. All-cause mortality was 27% over a median follow-up of sixty-two months (IQR 32–113). No distinguishable predictors of survival at presentation were found. Conclusions RA-OB appears to have a stable clinical course in the majority of patients despite persistent symptoms and severe obstruction based on presenting FEV1.