Long-term management challenges in postmyopathic dermatomyositis.

A 35-year-old woman was initially diagnosed with classic dermatomyositis (DM) at age 21. We cared for her during 11 of those 14 years. Traditional immunosuppressive treatments did not control her DM skin disease activity. She was subsequently observed to have a juvenile-onset DM phenotype. For the past 11 years, her skin and muscle disease activity had been only partially controlled with monthly high-dose intravenous immunoglobulin (IVIG) therapy. During those 11 years of IVIG therapy, her skin disease activity continued to flare intermittently, and she developed cutaneous calcinosis. Both refractory skin disease activity and cutaneous calcinosis are known poor prognostic indicators for DM patients. Preliminary evidence suggests that the oral targeted synthetic Janus kinase inhibitor, tofacitinib, can provide more complete suppression of DM disease activity and possibly reverse cutaneous calcinosis. Furthermore, better suppression of DM disease activity could lower the patient's risk of future DM comorbidities, such as premature atherosclerotic cardiovascular disease. We propose transitioning the patient from monthly high-dose IVIG to daily oral tofacitinib therapy. Another potential benefit for both the patient and society could be substantial healthcare cost savings.