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[炎症性肌病在临床实践中的重要性]

[Importance of inflammatory myopathies in practice].

作者信息

Pongratz D

机构信息

Friedrich-Baur-Institut der Medizinischen Fakultät an der Neurologischen Klinik, Ludwig-Maximilians-Universität München.

出版信息

Schmerz. 2003 Dec;17(6):432-6. doi: 10.1007/s00482-003-0255-5.

DOI:10.1007/s00482-003-0255-5
PMID:14648316
Abstract

Inflammatory myopathies can be subdivided into two main groups: infectious (bacterial, viral or other) myositis and immunogenic myositis. According to their frequency there are four main groups of diseases: dermatomyositis (DM), overlap syndromes, inclusion body myositis (IBM), idiopathic polymyositis (PM). Muscle weakness and atrophy are the prominent clinical symptoms of all immunogenic inflammatory myopathies. Muscle pain is not so common and occurs more frequently in acute forms than in chronic cases. Primary chronic forms of polymyositis and inclusion body myositis are mostly painless. Diagnosis starts with electromyography and laboratory investigations (especially CK). Myositis associated antibodies are mostly associated with acute forms. Radiological methods, especially MRI, are important in chronic cases. Definite diagnosis has to be done by muscle biopsy. Immunohistological (especially in DM and PM) and ultrastructural (especially in IBM) techniques are necessary in some cases. Concerning therapy of DM and PM, corticosteroids in combination with immunosuppressive drugs are effective in most cases. Intravenous immunoglobulins are needed only in selected cases (severe forms), side effects of therapy or resistance to conventional therapy. In IBM, it is the only therapeutic possibility, especially in young patients with rapid progression of the disease.

摘要

炎性肌病可分为两大类

感染性(细菌、病毒或其他)肌炎和免疫性肌炎。根据发病频率,有四大类疾病:皮肌炎(DM)、重叠综合征、包涵体肌炎(IBM)、特发性多发性肌炎(PM)。肌无力和萎缩是所有免疫性炎性肌病的突出临床症状。肌肉疼痛并不常见,在急性形式中比在慢性病例中更频繁出现。原发性慢性形式的多发性肌炎和包涵体肌炎大多无痛。诊断始于肌电图和实验室检查(尤其是肌酸激酶)。肌炎相关抗体大多与急性形式相关。放射学方法,尤其是磁共振成像(MRI),在慢性病例中很重要。明确诊断必须通过肌肉活检。在某些情况下,免疫组织学(尤其是在皮肌炎和多发性肌炎中)和超微结构(尤其是在包涵体肌炎中)技术是必要的。关于皮肌炎和多发性肌炎的治疗,皮质类固醇与免疫抑制药物联合在大多数情况下有效。仅在特定病例(严重形式)、治疗副作用或对传统治疗耐药时才需要静脉注射免疫球蛋白。在包涵体肌炎中,这是唯一的治疗选择,尤其是在疾病进展迅速的年轻患者中。

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Schmerz. 2003 Dec;17(6):432-6. doi: 10.1007/s00482-003-0255-5.
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本文引用的文献

1
High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study.散发性包涵体肌炎的大剂量免疫球蛋白治疗:一项双盲、安慰剂对照研究。
J Neurol. 2000 Jan;247(1):22-8. doi: 10.1007/s004150050005.
2
A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis.大剂量静脉注射免疫球蛋白输注治疗皮肌炎的对照试验。
N Engl J Med. 1993 Dec 30;329(27):1993-2000. doi: 10.1056/NEJM199312303292704.