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

Abstract

[...]its longitudinal progression should be further evaluated for appropriate management and counseling of patients. Table 3 Treatment and Outcome Directed therapy (N = 32, 78.0%) Long-acting inhaler, N (%) 27 (84.4) Systemic corticosteroids, N (%) 10 (31.3) Macrolides, N (%) 18 (56.3) Oxygen supplementation at rest Less than or equal to 3 L O2, N (%) 4 (12.5) Greater than 3 L, N (%) 1 (3.1) Transplantation, N (%) 1 (3.1) Mortality, N (%) 11 (26.8) Time from OB diagnosis to death, months (mean ± SD) 54 ± 53 Table 4 Univariable Cox regression predictors of all-cause mortality Characteristics HR (95% CI) P-value Age at diagnosis, years 0.99 (0.94–1.05) 0.78 Gender, female 0.93 (0.15–17.6) 0.94 Duration of RA prior to diagnosis, years 0.98 (0.94–1.03) 0.58 Active joint symptoms at diagnosis 1.87 (0.24–11.42) 0.51 Mosaic attenuation at diagnosis 0.59 (0.12–2.17) 0.44 Lung fibrosis at diagnosis 2.12 (0.58–7.76) 0.24 Bronchiectasis at diagnosis 1.02 (0.28–3.69) 0.97 Baseline PFTs at diagnosis TLC% 0.99 (0.95–1.04) 0.76 RV/TLC% 1.01 (0.98–1.03) 0.51 FVC% 0.94 (0.87–1.03) 0.19 FEV1% 0.99 (0.91–1.07) 0.96 Positive bronchodilator response 3.41 (0.73–17.7) 0.11 Macrolide therapy 1.73 (0.40–7.44) 0.44 Abbreviations: TLC% percent of predicted total lung capacity, RV/TLC% percent of predicted residual volume over total lung capacity, FVC% percent of predicted forced vital capacity, FEV1% percent of predicted forced expiratory volume in the first second, RA rheumatoid arthritis Discussion To our knowledge, this study represents the largest case series to date of patients with rheumatoid arthritis-associated obliterative bronchiolitis. Patients also had physiologic studies measured at varied intervals over time which make accurate assessment of rates of decline less reliable over the study interval. Because of the lack of standardized therapy, we were unable to make inferences about the efficacy of any particular treatment regimen on pulmonary function testing or clinical course. [...]as a retrospective study, exact circumstances involved in decision making are difficult to determine, particularly causal effects of management.