Neuroimmunology Unit, University of Athens Medical School, Athens, Greece.
Curr Opin Neurol. 2011 Oct;24(5):457-62. doi: 10.1097/WCO.0b013e32834a9589.
The inflammatory myopathies include polymyositis, dermatomyositis, necrotizing autoimmune myopathy (NAM), and inclusion body myositis (IBM). On the basis of clinical experience, most patients respond to corticosterioids to some degree or for a time period. For patients insufficiently responding or for steroid-sparing, the treatment options vary among practitioners, generating a genuine uncertainty. This timely review highlights emerging new therapies and provides practical therapeutic algorithms.
For patients insufficiently responding to corticosteroids, the commonly used immunosuppressants, such as azathioprine, mycophenolate, methotrexate, or cyclosporine, may exert a nonevidence-based 'steroid-sparing' effect but provide minimal benefit on their own. The second line therapy is intravenous immunoglobulin (IVIg) based on a controlled study conducted in dermatomyositis; the drug is also effective in many patients with polymyositis and NAM. Rituximab and tacrolimus may offer additional benefit. Anti-TNF agents are disappointing. IBM remains difficult to treat; although early on some patients may partially respond to steroids or IVIg, they soon become unresponsive and the disease progresses. Emerging agents against T cells, B cells, and transmigration molecules are discussed as promising therapeutic options.
New biological agents are in the offing for control trials. Appropriate outcome measures are however needed to assess and monitor responses.
炎症性肌病包括多发性肌炎、皮肌炎、坏死性自身免疫性肌病（NAM）和包涵体肌炎（IBM）。基于临床经验，大多数患者在某种程度上或在一定时间内对皮质类固醇有反应。对于反应不足或需要激素减量的患者，治疗选择因从业者而异，这确实存在不确定性。这篇及时的综述强调了新兴的治疗方法，并提供了实用的治疗算法。
对于对皮质类固醇反应不足的患者，常用的免疫抑制剂，如硫唑嘌呤、霉酚酸酯、甲氨蝶呤或环孢素，可能会产生一种无证据支持的“激素减量”作用，但自身益处有限。二线治疗是基于皮肌炎的对照研究的静脉注射免疫球蛋白（IVIg）；该药在许多多发性肌炎和 NAM 患者中也有效。利妥昔单抗和他克莫司可能会提供额外的益处。抗 TNF 药物令人失望。IBM 仍然难以治疗；尽管早期一些患者可能对类固醇或 IVIg 有部分反应，但很快就会变得无反应，疾病会进展。针对 T 细胞、B 细胞和迁移分子的新兴药物被讨论为有前途的治疗选择。
新的生物制剂即将进行对照试验。然而，需要适当的疗效评估指标来评估和监测反应。
