title: Developments in the classification and treatment of the juvenile idiopathic inflammatory myopathies.
abstract: This review updates recent trends in the classification of the juvenile idiopathic inflammatory myopathies (JIIM) and the emerging standard of treatment of the most common form of JIIM, juvenile dermatomyositis. The JIIM are rare, heterogeneous autoimmune diseases that share chronic muscle inflammation and weakness. A growing spectrum of clinicopathologic groups and serologic phenotypes defined by the presence of myositis autoantibodies are now recognized, each with differing demographics, clinical manifestations, laboratory findings, and prognoses. Although daily oral corticosteroids remain the backbone of treatment, disease-modifying anti-rheumatic drugs are almost always used adjunctively and biologic therapies may benefit patients with recalcitrant disease.
authors.affiliation: Environmental Autoimmunity Group, Program of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, CRC 4-2352, MSC 1301, 10 Center Drive, Bethesda, MD 20892-1301, USA; Myositis Center, Division of Rheumatology, Department of Medicine, George Washington University, G-400, 2150 Pennsylvania Avenue Northwest, Washington, DC 20037, USA. Electronic address: riderl@mail.nih.gov.
authors.forename: Lisa G
authors.initials: LG
authors.lastname: Rider
authors.forename: James D
authors.initials: JD
authors.lastname: Katz
authors.forename: Olcay Y
authors.initials: OY
authors.lastname: Jones
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D002648:Child; D016572:Cyclosporine / Q000627:therapeutic use; D003882:Dermatomyositis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D009220:Myositis / Q000188:drug therapy / Q000276:immunology*; D011241:Prednisone / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011241:Prednisone; D008775:Methylprednisolone; D008727:Methotrexate
keywords: Biologic therapies; Juvenile dermatomyositis; Juvenile myositis; Juvenile polymyositis; Myositis-specific autoantibodies; Overlap myositis; Treatment