Case report: Successful treatment of anti-MDA5-positive to negative dermatomyositis-associated interstitial lung disease with the JAK inhibitor tofacitinib

ObjectiveAnti-MDA5 antibody-positive dermatomyositis (DM) is a rare clinical autoimmune disease, and anti-MDA5-positive DM with interstitial lung disease (ILD) is the most important cause of death in DM patients. We reported the efficacy of the JAK1/3 inhibitor tofacitinib as an anti-MDA5-negative treatment option for patients with anti-MDA5-positive DM-ILD. Method and processHere we report a 51-year-old female patient with cough, sputum, shortness of breath for 5 months, rash for 3 months, and muscle pain in the extremities for 1 month. After conventional immunosuppressive therapy plus hormone therapy, the remission was slow. Methylprednisolone was successfully reduced after we administered tofacitinib and tacrolimus. After 132 weeks of follow-up, anti-MDA5 antibody turned negative, clinical symptoms were relieved, and lung Imaging tests were successfully reversed. Results and ConclusionThere is currently no report of tofacitinib supplement therapy for anti-MDA5 positive to negative DM. With this case report, tofacitinib is an option for the treatment of anti-MDA5-positive DM-ILD, which deserves attention.

Automated summary

This study reports a case of anti-MDA5 antibody-positive DM-ILD, in which tofacitinib and tacrolimus were used as treatment options after conventional immunosuppressive therapy and hormone therapy. The patient showed remission of clinical symptoms, reversal of lung imaging tests, and conversion of anti-MDA5 antibody from positive to negative.