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Interstitial lung disease (ILD) is associated with increased morbidity and mortality in rheumatoid arthritis (RA). Moreover, acute exacerbation (AE) is a devastating complication of RA plus ILD. However, few data on AE in RA-associated ILD are available. ⋯ Our findings suggest that approximately one-third of patients with RA-associated ILD experience AE and that ever smoker status, and lower lung function and exercise capacity predispose patients to AE. AE significantly affects the overall survival of patients with RA-associated ILD.