Postolova Anna, Chen Jennifer K, Chung Lorinda
Division of Rheumatology and Immunology, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA.
Department of Dermatology, Stanford Hospital and Clinics, 450 Broadway Street, Pavilion C, Suite 242, Redwood City, CA 94063, USA.
Rheum Dis Clin North Am. 2016 Feb;42(1):103-18, ix. doi: 10.1016/j.rdc.2015.08.011. Epub 2015 Oct 26.
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.
特发性炎性肌病（IIMs）累及肌肉炎症，根据临床表现模式以及皮肤和肌肉活检的免疫组织病理学特征分为4类：皮肌炎、多发性肌炎、包涵体肌炎和免疫介导的坏死性肌病。全身使用糖皮质激素（CS）治疗是有肌肉和器官受累的IIM的标准治疗方法。系统性硬化症的皮肤外表现常采用CS治疗；然而，高剂量CS与高危患者的硬皮病肾危象有关。尽管CS可以作为有效的一线药物，但其显著的副作用促使人们联合使用其他免疫抑制药物以便及时减药。
