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[炎性肌病:皮肌炎、多发性肌炎和包涵体肌炎]

[Inflammatory muscle diseases: dermatomyositis, polymyositis, and inclusion body myositis].

作者信息

Genth E

机构信息

Rheumaklinik und Rheumaforschungsinstitut Aachen.

出版信息

Internist (Berl). 2005 Nov;46(11):1218-32. doi: 10.1007/s00108-005-1496-4.

Abstract

Dermatomyositis, polymyositis, inclusion body myositis and myositis overlap syndromes are systemic immune disorders of unknown origin with muscle weakness and elevated values of creatinkinase in the serum. Muscle biopsy is pivotal for a proper clinical diagnosis. Extramuscular findings at the skin, the joints or internal organs (lung, heart) are characteristic for the different clinical presentations of dermato- or polymyositis and are usually absent in inclusion body myositis. With the exception of inclusion body myositis myositis-associated autoantibodies are frequently present and associated with distinct clinical manifestations (e. g. antisynthetase syndrome). The rate of malignancy is elevated for several years after onset of myositis. Especially in polymyositis an appropriate differential diagnosis of infectious, endocrine, metabolic or neuromuscular causes of muscle disease is necessary. Glucocorticosteroids are the first choice of treatment in dermato- or polymyositis. Methotraxate, azathioprine, cyclophosphamamide, i.v. immunoglobulins and other drugs are used in diseases courses with continuous high dose requirement of corticosteroids.

摘要

皮肌炎、多发性肌炎、包涵体肌炎和肌炎重叠综合征是病因不明的全身性免疫疾病,伴有肌肉无力和血清肌酸激酶值升高。肌肉活检对正确的临床诊断至关重要。皮肤、关节或内脏器官(肺、心脏)的肌肉外表现是皮肌炎或多发性肌炎不同临床表现的特征,而在包涵体肌炎中通常不存在。除包涵体肌炎外,肌炎相关自身抗体经常出现,并与不同的临床表现相关(例如抗合成酶综合征)。肌炎发病后的几年里,恶性肿瘤发生率会升高。特别是在多发性肌炎中,有必要对肌肉疾病的感染、内分泌、代谢或神经肌肉病因进行适当的鉴别诊断。糖皮质激素是皮肌炎或多发性肌炎治疗的首选。甲氨蝶呤、硫唑嘌呤、环磷酰胺、静脉注射免疫球蛋白和其他药物用于需要持续高剂量使用糖皮质激素的病程中。

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