Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients. Although CS can be effective first-line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering.
Key points
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Treatment with corticosteroids is the standard of care for idiopathic inflammatory myopathies with muscle and organ involvement.
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Extracutaneous features of scleroderma occasionally warrant treatment with corticosteroids; however, high doses have been associated with the development of scleroderma renal crisis in high-risk patients.
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Corticosteroid monotherapy is not recommended for cutaneous manifestations of dermatomyositis and scleroderma.
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The significant side effect profile of corticosteroids encourages concomitant treatment with other immunosuppressive medications to enable timely tapering.
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