Calcinosis in refractory dermatomyositis improves with tofacitinib monotherapy: a case series
Matthew Shneyderman, Shivani Ahlawat, Lisa Christopher‐Stine, Julie J. Paik
Abstract
Dear Editor, We report three patients with refractory DM and calcinosis who were part of an open-label clinical trial [Study of Tofacitinib in Refractory Dermatomyositis (STIR)] [1] who also had improvement in their calcinosis that coincided with improvement on muscle MRI after 3 months of treatment. Minimum Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) of >5 and/or Manual Muscle Testing-8 (MMT-8) <142 was required to enrol in the study. This study was approved by the Johns Hopkins Institutional Review Board and each patient gave written informed consent. Patient 1 is a 50-year-old female with a history of NXP-2 positive refractory DM for 14 years prior to enrolment in the STIR study. Her initial presentation was manifested by proximal muscle weakness and dysphagia and classic rash including Gottron’s papules and V-neck sign. She developed calcinosis of the left calf within 3 months of the onset of her DM, which progressed to include her thighs. While she had improvement in her muscle disease with immunosuppression, her continued skin disease activity prompted entry into the STIR study. There was an improvement in her skin disease with tofacitinib (see CDASI scores in Supplementary Table S1, available at Rheumatology online). Her muscle strength was full power at entry into the study. On imaging, there was elevated signal of the intramuscular and subcutaneous reticular areas on baseline bilateral thigh muscle MRI. After 3 months of treatment, the patient noticed an improvement in her calcinosis in the calf and thighs, which was also confirmed on muscle MRI (Fig. 1A–D).