无肌病性皮肌炎

Amyopathic dermatomyositis.

作者信息

Olsen N J, Park J H, King L E

机构信息

Division of Rheumatology, Department of Medicine, Vanderbilt University, T-3219 Medical Center North, Nashville, TN 37232, USA.

出版信息

Curr Rheumatol Rep. 2001 Aug;3(4):346-51. doi: 10.1007/s11926-001-0039-9.

Abstract

Amyopathic dermatomyositis is a variant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities. It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the observation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensitive measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although juvenile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur. Determining whether a patient has amyopathic rather than myopathic disease may have prognostic implications.

摘要

无肌病性皮肌炎是皮肌炎的一种变体,其特征为有典型的皮疹但无肌肉异常。有人提出,无肌病性和肌病性皮肌炎处于连续统一体中,这一概念得到了家族和遗传学研究以及以下观察结果的支持:一小部分无肌病性患者会转变为明显的肌病状态。无肌病状态通过缺乏肌无力以及包括血清肌酶、肌电图研究和肌肉活检在内的诊断测试来定义,这些测试通常正常或仅显示轻微异常。尽管没有肌无力,但许多患者抱怨极度疲劳。更敏感的肌肉功能测量方法,如磷-31磁共振波谱分析,表明无肌病性患者的肌肉代谢异常。无肌病形式在成人中比在儿童中更常见,尽管也有青少年病例的报道。一些早期系列研究表明与潜在恶性肿瘤无关,但最近的报告表明会发生恶性肿瘤。确定患者患有无肌病性而非肌病性疾病可能具有预后意义。

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