Consensus treatments for moderate juvenile dermatomyositis: Beyond the first two months. Results of the Second Childhood Arthritis and Rheumatology Research Alliance Consensus Conference

Juvenile dermatomyositis (JDM) is a rare, autoimmune illness characterized by muscle and skin involvement, with less frequent involvement of other systems, including the gastrointestinal tract and lungs. Although previously associated with significant mortality (1), morbidity is a much greater problem since the introduction of corticosteroids as a mainstay of therapy. Many children experience complications of their underlying disease such as contractures, weakness, disfiguring skin lesions and painful calcinosis. They may also develop complications secondary to prolonged courses of treatment, in particular those side effects related to chronic corticosteroid use(2). There is very little data on which to base treatment decisions in JDM. Even the most commonly used therapies, corticosteroids and/or methotrexate, have not been studied in clinical trials. In fact, at the time of this writing, no randomized clinical trials of therapy in JDM have been published. This lack of data has resulted in wide variation in treatment of children with JDM. This has been documented by Stringer et al. who reported the results of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM treatment survey (3). Using a series of representative clinical scenarios of children with JDM, members of CARRA were surveyed with regard to investigations and therapies. The survey was answered by 141 CARRA members (84% response rate), and revealed a remarkable degree of variation in dose, duration and route of corticosteroids, and use of methotrexate and other immunosuppressive agents (3). In 2007, 12 pediatric rheumatologists experienced in the care of children with JDM participated in a CARRA-initiated consensus conference in Toronto, Ontario, Canada (4). This meeting had the explicit goal of defining a small number of consensus treatment plans for the initial treatment (up to 2 months) of children with moderate JDM. Using data from the CARRA JDM treatment survey (3) and expert opinion, the meeting participants were able to develop 3 consensus treatment plans. It is important to note that these treatment plans were not intended to be innovative. Rather, the goal was to develop treatment plans which were similar to approaches that were being used commonly in the pediatric rheumatology community. It was hoped that by developing consensus treatment plans, variation in treatment approaches could be decreased and data could be prospectively collected. This would allow for comparative research and would be the first step in defining evidence-based treatments for moderate JDM. After the success of the Toronto consensus meeting, it was recognized that for these treatment plans to be studied, they needed to be extended beyond 2 months of treatment. The goal of the present effort was to use consensus methods, and the considerable expertise contained within the CARRA organization, to extend the 3 previously developed treatment plans to span the full course of treatment of children with moderate JDM.