Tuesday, 07 Apr 2020

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Challenges of Dermatomyositis Progress

In the current issue of Lancet, Pierre Miossec discusses the challenges of studying and managing juvenile dermatomyositis and, specifically, the PRINTO trial (previously reported herein). This study demonstrated that combining prednisone with either cyclosporine (CyA) or methotrexate (MTX) yielded the best efficacy, but the safety profile and steroid-sparing effect favored the combination of Pred + MTX combination most.

The consequences of dermatomyositis, with inflammatory myopathy and skin manifestations over the face and metacarpophalangeal joints, are well known and include: 1) muscle inflammation and damage; 2) rising muscle enzymes; 3) circulating autoantibodies; and 4) the need for high dose corticosteroids and immunosuppressives.

The PRINTO trial compared three drug regimens in 139 children: A) prednisone alone, B) prednisone plus MTX or C) prednisone plus CyA (n=46). Responses favored predisone with either MTX or CyA (with 70-72% responding) and the time to discontinuation of prednisone shorter with DMARDs (30 mos vs 36 mos).

One of the problems he notes is that it took 54 centres from 22 countries to enroll fewer than 150 patients over 4 years. Delays in care, involvement of many different specialists and variations in practice amplify the challenges of doing a proper trial in this disorder. Nonetheless, the PRINTO trial is testimony to the fact that such trials can be done and are needed to further our understanding and treatment of juvenile dermatomyositis.


The author has no conflicts of interest to disclose related to this subject

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