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[进行性系统性硬化症肌肉组织的免疫形态学和超微结构研究结果]

[Immunomorphological and ultrastructural findings on the musculature in progressive scleroderma].

作者信息

Klug H, Barthelmes H, Thormann T

出版信息

Z Gesamte Inn Med. 1977 Aug 1;32(15):357-63.

PMID:335686
Abstract

Muscle biopsies taken from 30 patients with generalized scleroderma were studied immuno-histologically as well as by light and electron microscopy. The immunological investigations have demonstrated that an increase of IgM and/or complement depositions in capillaries and in the walls of small arteries have been involved in most cases. Similar alterations were found in the subsarcolemmal zone. Vascular Ig-G precipitates were observed in the later stage of the disease only, whereas a positive reaction of fibrinogen have been proved in the most cases. By light and electron microscopy a variety of pathologic alterations have usually been found, but the most prominent changes involved were thickening of the capillary basement membrane and interstitial fibrosis. Scattered cellular infiltrates consisting of lymphocytes, histiocytes and plasmocytes were also seen either perivascularly or throughout the interstice. Around fibroblasts and capillaries, fine protocollagen fibrils and glycosaminoglycan filaments have been observed. They may partly represent fibrogenesis. Filamentous structures could be seen within the cytoplasm of numerous fibroblasts.

摘要

对30例全身性硬皮病患者的肌肉活检标本进行了免疫组织学、光镜及电镜研究。免疫研究表明,大多数病例中,毛细血管和小动脉壁上IgM和/或补体沉积增加。在肌膜下区域也发现了类似改变。仅在疾病后期观察到血管Ig-G沉淀,而大多数病例中已证实纤维蛋白原有阳性反应。通过光镜和电镜通常发现了多种病理改变,但最显著的变化是毛细血管基底膜增厚和间质纤维化。在血管周围或整个间隙中也可见由淋巴细胞、组织细胞和浆细胞组成的散在细胞浸润。在成纤维细胞和毛细血管周围观察到细的原胶原纤维和糖胺聚糖丝。它们可能部分代表纤维生成。在许多成纤维细胞的细胞质内可见丝状结构。

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