Abstract
BackgroundRheumatoid 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.MethodsWe 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.ResultsForty 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.ConclusionsRA-OB appears to have a stable clinical course in the majority of patients despite persistent symptoms and severe obstruction based on presenting FEV1.
Highlights
Rheumatoid arthritis (RA) is a systemic autoimmune condition characterized by erosive inflammation of the joints
Obliterative bronchiolitis was defined using the following criteria: 1) presence of active compatible respiratory symptoms including dyspnea or cough plus 2) abnormal pattern on pulmonary function testing (PFT) in the absence of radiologic emphysema or 3) evidence of small airways disease on high-resolution computed tomography (CT) of the chest such as mosaicism or air trapping or centrilobular nodules or 4) histopathologic features on lung biopsy consistent with obliterative bronchiolitis (OB), when available in 5) the absence of an alternative diagnosis that could account for the findings
Airflow obstruction was defined as Forced expiratory volume in the first second (FEV1) to Forced vital capacity (FVC) ratio less than the lower limit of normal (LLN), with staging of obstructive disease based on GOLD criteria for FEV1 [22]
Summary
Rheumatoid arthritis (RA) is a systemic autoimmune condition characterized by erosive inflammation of the joints. Rheumatoid arthritis (RA) is a systemic autoimmune condition characterized primarily by erosive inflammation of the joints affecting approximately 1% of the world’s population [1] It is often associated with high morbidity due to extra-articular manifestations. Obliterative bronchiolitis (OB, synonymous with constrictive bronchiolitis) is a rare small airways disease characterized by the destruction of bronchiolar epithelium and subsequent progressive airflow obstruction [7] It has been found in a wide range of clinical settings. Due to its rarity, there is still a paucity of literature regarding the clinical course of rheumatoid arthritis-associated obliterative bronchiolitis (RA-OB) It has been previously described as variable, in that some patients have periods of prolonged stability while others have a more rapid decline with respiratory failure and death [7]
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