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混合性结缔组织病——告别这一切。

Mixed connective tissue disease--goodbye to all that.

作者信息

Black C, Isenberg D A

机构信息

Department of Rheumatology, Royal Free Hospital, London.

出版信息

Br J Rheumatol. 1992 Oct;31(10):695-700. doi: 10.1093/rheumatology/31.10.695.

Abstract

Since it was first described mixed connective tissue disease (MCTD) has been the subject of much debate. In particular the question of whether it is a truly distinctive disease entity has been challenged. It seems clear that the original description of MCTD as a mild disorder, rarely affecting the lungs or kidneys and requiring small doses of corticosteroids only, is no longer tenable. In this review a historical analysis of the clinical and serological features is presented. It is suggested that the concept of MCTD as a distinct disease entity is better replaced by the term 'undifferentiated autoimmune rheumatic/connective tissue disorder'. Many of these patients will later 'convert' into scleroderma or lupus; some will remain undifferentiated.

摘要

自混合性结缔组织病(MCTD)首次被描述以来,一直备受争议。尤其是它是否为一种真正独特的疾病实体这一问题受到了质疑。很明显,最初将MCTD描述为一种轻度疾病,很少影响肺部或肾脏,仅需小剂量皮质类固醇治疗的说法已不再成立。在本综述中,对其临床和血清学特征进行了历史分析。有人建议,将MCTD作为一种独特疾病实体的概念,最好用“未分化自身免疫性风湿性/结缔组织病”这一术语来取代。这些患者中的许多人后来会“转变”为硬皮病或狼疮;有些人则会一直保持未分化状态。

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