Successful dose escalation of tofacitinib for refractory dermatomyositis and interstitial lung disease with anti-melanoma differentiation-associated gene 5 antibodies

Abstract Anti-melanoma differentiation-associated gene 5 (MDA-5) antibodies have widely known to be associated with amyopathic dermatomyositis with rapidly progressive interstitial lung disease (ILD). Although the triple combination therapy with high-dose glucocorticoids, cyclophosphamide, and a calcineurin inhibitor has been used to treat anti-MDA-5 antibody-positive rapidly progressive ILD, the prognosis of these patients remains poor despite this intensive therapy. Recently, several investigators have shown that combination therapy with tofacitinib might be potentially efficacious in those patients. We herein report a case of anti-MDA-5 antibody-positive dermatomyositis and associated ILD who had not responded to the triple therapy and tofacitinib 10 mg/day but markedly responded after increasing the dose of tofacitinib to 20 mg/day.