Developments in the classification and treatment of the juvenile idiopathic inflammatory myopathies
2013
The juvenile idiopathic
inflammatory myopathies(JIIM) are rare, heterogeneous autoimmune diseases that share chronic
muscle inflammationand weakness. JIIM broadly includes three major clinicopathologic groups:
juvenile dermatomyositis, juvenile
polymyositis, and overlap
myositis. A growing spectrum of clinicopathologic groups and serologic phenotypes defined by the presence of
myositis-specific or
myositis-associated autoantibodies are now recognized, each with differing demographics, clinical manifestations, laboratory findings, and prognoses. With the first multi-center collaborative studies and controlled trials using standardized preliminarily validated outcome measures, the therapy of juvenile
myositishas advanced. Although daily oral corticosteroids remain the backbone of treatment, disease-modifying anti-rheumatic drugs (DMARDs) are almost always used as adjunctive therapy.
Methotrexateis the conventional DMARD for the initial therapy, either alone or combined with intravenous pulse
methylprednisolone, and/or intravenous immunoglobulin for patients with moderate to severe disease. Cyclosporine may be added to these or serve as an alternative to
methotrexate. Other drugs and biologic therapies, including
mycophenolatemofetil,
tacrolimus, cyclophosphamide, rituximab, and
infliximab, might benefit selected patients with recalcitrant disease, unacceptable steroid toxicity, or patients with risk factors for poor prognosis. The treatment of cutaneous disease,
calcinosis, and the role for rehabilitation are also discussed.
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