Cyclosporin A and intravenous immunoglobulin treatment in polymyositis/dermatomyositis.

Abstract

Introduction: Corticosteroids remain the agents of choice for the initial empiric treatment of inflammatory myopathy, but many patients will require the addition of a non-steroidal immunosuppressive (IS) drug to control myositis or its extramuscular features. Multiple IS drugs have been used including methotrexate, azathioprine, cyclosporine, and mycophenolate mofetil. Alkylating agents, such as cyclophosphamide and chlorambucil, and novel immunosuppressives, such as tacrolimus and fludarabine, have also been studied in controlledand openprospective fashion. Numerous combination IS regimens are being reported, and intravenous immune globulin (IVIg) has been shown to be beneficial in a wide variety of diseases with a suspected immunologic basis, including myositis. The difficulty with assessing the treatment of idiopathic inflammatory myopathy (IIM) relates to many factors. The disease is rare and quite heterogeneous leading to a paucity of well-controlled prospective trials. Most studies are cross-sectional in nature and involve single centers with a relatively small number of patients. Treatment trials often include patients with acute and chronic disease, creating the dilemma of determining the relative contributions of potentially reversible disease activity versus irreversible damage, which is unlikely to respond to therapeutic intervention. To complicate matters further, agreed-on outcome measures have yet to be developed and tested.