Interestingly, the anti-fibrotic effects of selective JAK2 inhibitors on fibroblasts and in models for pulmonary fibrosis decreased over time because of reactivation of JAK2 by induction of JAK1.75 In human studies, a single-arm open-label trial of 18 patients revealed that tofacitinib demonstrates greater survival in patients with amyopathic dermatomyositis-ILD compared to conventional treatment.76 A retrospective study evaluated the efficacy of tofacitinib in patients with dermatomyositis-ILD resistant to triple therapy (high-dose glucocorticoid, cyclosporine A, and cyclophosphamide). This evidence concerns the gene JAK2 and interstitial lung disease.