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Tofacitinib in Amyopathic Dermatomyositis–Associated Interstitial Lung Disease

2019; Massachusetts Medical Society; Volume: 381; Issue: 3 Linguagem: Inglês

10.1056/nejmc1900045

1533-4406

Zhiwei Chen, Xiaodong Wang, Shuang Ye,

Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

Tofacitinib in Amyopathic Dermatomyositis-Associated Interstitial Lung DiseaseTo the Editor: Anti-melanoma differentiationassociated protein (MDA) 5 (anti-MDA5)-positive amyopathic dermatomyositis (ADM)-associated interstitial lung disease (ILD) (hereafter, ADM-ILD) is a rapidly progressive and life-threatening disease. 1 Despite aggressive conventional treatments with high-dose glucocorticoids in combination with cyclosporine, tacrolimus, or cyclophosphamide, the 6-month mortality is as high as 50%. 2,3Japanese investigators recently reported the possible efficacy of tofacitinib, a Janus kinase (JAK) inhibitor, as a rescue option for patients with high-risk ADM-ILD after failure of conventional treatment. 3 Our data have also suggested that a JAK inhibitor could abrogate the proinflammatory and profibrotic effects of ADM-ILDderived T cells in vitro. 4 Thus, we conducted a single-center, open-label clinical study (Chinese Clinical Trial Registry number, ChiCTR-1800016629) to evaluate the efficacy of tofacitinib in patients with early-stage anti-MDA5positive AMD-ILD.Patients were included in the trial if they were older than 18 years of age, met the criteria for a diagnosis of ADM 2 and were positive for anti-MDA5 antibodies, had had ILD (confirmed by pulmonary high-resolution computed tomography [CT]) for less than 3 months, and had a predicted forced vital capacity (FVC) of at least 50%.Patients were excluded if they had other connective-tissue diseases, an underlying cancer, a concomitant infection, or a liver aminotransferase level greater than 2 times the upper limit of the normal range.From July 2017 through September 2018, a total of 18 consecutive patients who were eligible to participate in the trial were prospectively enrolled at our center and received a glucocorticoid