Abstract

Interstitial lung disease (ILD) is one of the most frequent and severe visceral complications of connective tissue diseases (CTDs).1 Despite the recent approval of nintedanib for the treatment of fibrosing progressive ILDs other than idiopathic pulmonary fibrosis,2 management of CTD-ILDs remains challenging. While fibrosing ILD represents at least 50% of ILD cases in patients with rheumatoid arthritis, the frequency of fibrosing subsets in CTDs is significantly lower.1,3 Therefore, immunosuppressants continue to be the most commonly adopted therapy for CTD-ILDs.

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