Abstract

Interstitial lung disease (ILD) associated with idiopathic inflammatory myopathy is a life-threatening organ involvement [1–4]. Particularly, anti-melanoma differentiation-associated gene 5 (MDA5) antibody is associated with rapid progressive and refractory ILD [1,2], and the prognosis of patients with anti-MDA5-positive ILD is extremely poor, with the mortality of 31.7–45.0% [2,5]. Thus, establishment of optimal treatment strategy is an urgent task. Recently, the effectiveness of combined immunosuppressive regimen consisting of high-dose glucocorticoids, calcineurin inhibitors, and intravenous cyclophosphamide treatment [6], rituximab [7,8] and plasma exchange [9] have been reported, however, many patients have still failed to respond to those treatment resulting in a fatal consequence. Tofacitinib is a Janus kinase (JAK) inhibitor approved for rheumatoid arthritis, ulcerative colitis, and atopic dermatitis. It inhibits JAK1, JAK3, and, a lesser extent, JAK2 [10]. Tofacitinib has been...

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