Adult inflammatory myopathies
Adult inflammatory myopathies
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DOI：
10.1016/j.berh.2004.02.009
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发表时间：
2004-06-01
影响因子：
5.2
通讯作者：
Plotz, PH
中科院分区：
文献类型：
--
作者：
Christopher-Stine, L;Plotz, PH
成人的主要炎症性肌病-皮肌炎、多发性肌炎和包涵体肌炎-是不常见的，很难与许多临床上模拟它们的情况区分开来。它们的发病率很高；它们经常是相关恶性肿瘤的第一个征兆；它们可能是另一种结缔组织疾病的一部分。它们的致病特征表明它们是不同的疾病。皮肌炎和多发性肌炎在临床和组织学上都是明显的炎症性疾病，通常对针对炎症的治疗都有反应。包涵体肌炎现在被普遍认为是50岁以上患者最常见的肌病，它对免疫抑制治疗的反应很温和，有时根本不起作用。本文就其发病机制、肌外器官受累、鉴别诊断、诊断方法和治疗主线等方面的新认识作一综述。
The major inflammatory myopathies of adults-dermatomyositis, polymyositis and inclusion body myositis-are uncommon and can be difficult to distinguish from many conditions that mimic them clinically. They have a high morbidity; they are not infrequently the first sign of an associated malignancy; and they may be a part of another connective tissue disease. Their pathogenetic features suggest that they are different illnesses. Dermatomyositis and polymyositis are clearly inflammatory, both clinically and histologically, and both generally respond to therapy directed towards inflammation. Inclusion body myositis is now generally recognized as the most common myopathy presenting in patients over the age of 50 years, and it responds only modestly and sometimes not at all to immunosuppressive therapy. In this review, we have summarised the major newly recognized features of pathogenesis, the involvement of extramuscular organs, the differential diagnosis, diagnostic approaches and the main lines of therapy.