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[一例成人多发性肌炎中的束周坏死与再生]

[Perifascicular necrosis and regeneration in a case of adult polymyositis].

作者信息

Iwatsubo T, Kimura T, Inoue K

机构信息

Department of Neurology, Faculty of Medicine, University of Tokyo.

出版信息

Rinsho Shinkeigaku. 1991 Apr;31(4):439-44.

PMID:1914331
Abstract

We report a case of adult polymyositis with peculiar muscular pathology of innumerable muscle fiber necrosis and regeneration accentuated in the perifascicular area. A 51-year-old woman developed generalized weakness of the extremities, trunk and bulbar muscles subacutely for two months. Anterior tibial muscle biopsy showed numerous tube-like necrotic/regenerative muscle fibers predominantly in the perifascicular area. The diameters of the muscle fibers were smaller in the periphery of the fascicles. Small arteries at the center of the fascicles occasionally showed marked perivascular cuffing, although complement component C9 was negative in the vessel wall. The gradient of the diameters of necrosis/regeneration fibers was thought to have been caused by ischemia of the muscles, which persisted at the perifascicular area leading to recurrent necrosis and regeneration and gradually invaded towards the center of the fascicles. We designated this muscular pathology as perifascicular necrosis and regeneration, and regarded it was an acute severe form of the perifascicular ischemic lesions of myositis.

摘要

我们报告一例成人多发性肌炎,其具有特殊的肌肉病理学表现,即无数肌纤维坏死和再生,在束周区域更为明显。一名51岁女性在两个月内亚急性出现四肢、躯干和延髓肌肉的全身性无力。胫前肌活检显示,主要在束周区域有大量管状坏死/再生肌纤维。肌束周边的肌纤维直径较小。尽管血管壁中的补体成分C9呈阴性,但肌束中心的小动脉偶尔会出现明显的血管周围套袖样改变。坏死/再生纤维直径的梯度变化被认为是由肌肉缺血引起的,这种缺血在束周区域持续存在,导致反复坏死和再生,并逐渐向肌束中心蔓延。我们将这种肌肉病理学表现命名为束周坏死和再生,并认为它是肌炎束周缺血性病变的一种急性严重形式。

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