Patients with interstitial pneumonia with autoimmune features (IPAF) have a 14-fold higher risk of progressing to a rheumatic disease than patients with interstitial lung disease (ILD) but not IPAF, according to the results of a retrospective cohort study. The term IPAF is used to describe individuals with ILD who have some features of autoimmunity, but who do not fulfil the classification criteria for an autoimmune rheumatic disease. Of the 174 patients included in the study, 8 out of 50 patients with IPAF (16%) developed rheumatic diseases after a median of 5.2 years, compared with 2 out of 124 patients with ILD (1.6%).
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Alevizos, M. K. et al. Risk of progression of interstitial pneumonia with autoimmune features to a systemic autoimmune rheumatic disease. Rheumatology https://doi.org/10.1093/rheumatology/kez404 (2019)
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Collison, J. Increased risk of rheumatic disease in IPAF. Nat Rev Rheumatol 15, 698 (2019). https://doi.org/10.1038/s41584-019-0327-2
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DOI: https://doi.org/10.1038/s41584-019-0327-2
