Myositis Center, Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
J Intern Med. 2016 Jul;280(1):63-74. doi: 10.1111/joim.12511. Epub 2016 Apr 20.
The management of patients with idiopathic inflammatory myopathy (IIM) remains a challenge given the systemic features beyond active myositis. That is, recognizing the inflammatory arthropathy, varying dermatomyositis rashes, and overt and occult features of interstitial lung disease in addition to myositis adds to the complexity of diagnosis and treatment of IIM. However, clinicians now have available many more immunosuppressive drugs as well as biologic agents for use in patients with myositis and other autoimmune diseases. Here, the use of these agents is reviewed and support based on available published literature is provided even though many studies have been small and results somewhat anecdotal. Glucocorticoids remain the initial treatment of choice in most instances and methotrexate and azathioprine are often used early in the treatment course. These agents are followed by other immunosuppressive drugs, for example mycophenolate mofetil, tacrolimus, cyclosporine and cyclophosphamide, some of which are used alone while combinations of these agents also provide an effective option. There is more rationale for the use of biologic agents such as rituximab from a mechanistic perspective and, given the incorporation of validated core set measures in assessing myositis patients, we can look forward to better designed clinical trials in the future.
由于特发性炎症性肌病 (IIM) 除了肌炎之外还具有全身特征,如炎症性关节病、不同的皮肌炎皮疹以及明显和隐匿性的间质性肺病特征,因此患者的管理仍然是一个挑战。此外,临床医生现在有更多的免疫抑制剂和生物制剂可用于治疗肌炎和其他自身免疫性疾病。在这里,我们回顾了这些药物的使用,并根据现有文献提供了支持,尽管许多研究规模较小,结果有些传闻。在大多数情况下,糖皮质激素仍然是首选的初始治疗药物,甲氨蝶呤和硫唑嘌呤通常在治疗早期使用。这些药物之后是其他免疫抑制剂,例如霉酚酸酯、他克莫司、环孢素和环磷酰胺,其中一些单独使用,而这些药物的组合也提供了有效的选择。从机制的角度来看,生物制剂如利妥昔单抗的使用更有道理,并且鉴于在评估肌炎患者时纳入了经过验证的核心集措施,我们可以期待未来有更好设计的临床试验。