德国关于皮肌炎的最新诊疗指南

目前的皮肌炎指南是德国神经学会跨学科S2K肌炎综合征指南的摘录。皮肌炎的肌炎症状主要是对称性近端肌无力，而CRP、ESR和肌酸激酶升高及肌电图和肌肉活检也可提供重要的诊断线索。咽、呼吸、心脏和颈部肌肉也可受到影响。鉴于约30%的患者有间质性肺病，故应进行肺功能试验。

虽然皮肌炎的皮肤表现多种多样，总体看来，它们构成了皮肌炎特征性和关键性的诊断标准。大约5％～20％的患者有典型的皮肤病变而无任何临床肌肉受累表现（即无肌病性皮肌炎）。虽然30％的成人皮肌炎患者伴发恶性肿瘤，但不应该耽误严重肌炎的治疗。

糖皮质激素是治疗肌炎的首选药（1-2mg/kg），常需要用其他免疫抑制（硫唑嘌呤，儿童用甲氨蝶呤）。如对治疗反应不好，可用静脉注射免疫球蛋白。利妥昔单抗的疗效还未最终确定。急性治疗后，通常继以低剂量维持治疗1~3年。

对肌炎有效的治疗不一定对皮肤病变有效，可外用糖皮质激素和钙调磷酸酶抑制剂，还可全身用抗疟药（可联合）、甲氨蝶呤和糖皮质激素，静脉注射免疫球蛋白或利妥昔单抗可能也有帮助。紫外线防护也是很重要的。

附原文

Summary  The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at www.awmf.org). The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30% of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup. Although the cutaneous manifestations in DM are variable, taken together, they represent a characteristic and crucial diagnostic criterion for DM. Approximately 5–20% of individuals exhibit typical skin lesions without any clinically manifest muscle involvement (amyopathic DM). About 30% of adult DM cases are associated with a malignancy. This fact, however, should not delay the treatment of severe myositis.Corticosteroids are the therapy of choice in myositis (1–2mg/kg). Additional immunosuppressive therapy is frequently required (azathioprine, for children methotrexate). In case of insufficient therapeutic response, the use of intravenous immunoglobulins is justified. The benefit of rituximab has not been conclusively ascertained yet. Acute therapeutic management is usually followed by low-dose maintenance therapy for one to three years. Skin lesions do not always respond sufficiently to myositis therapy. Effective treatment for such cases consists of topical corticosteroids and sometimes also calcineurin inhibitors. Systemic therapies shown to be effective include antimalarial agents (also in combination), methotrexate, and corticosteroids. Intravenous immunoglobulins or rituximab may also be helpful. UV protection is an important prophylactic measure.

引自：Sunderkötter C, Nast A, Worm M, et al. 2016 Guidelines on dermatomyositis – excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology. J Dtsch Dermatol Ges 2016,14(3):321-38