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硬皮病(进行性系统性硬化症)和雷诺病中毛细血管的超微结构变化及基底膜增厚

Fine structural capillary changes and basal lamina thickening in scleroderma (progressive systemic sclerosis) and Raynaud's disease.

作者信息

Camilleri J P, Fiessinger J N, Debure C, Bruneval P, Tricottet V, Kazandjian S, Housset E

出版信息

Pathol Res Pract. 1984 Jan;178(3):230-6. doi: 10.1016/S0344-0338(84)80104-1.

Abstract

Capillary abnormalities were found by electron microscopy in labial salivary gland biopsies obtained from 20 patients with progressive systemic sclerosis (PSS). They consisted of marked thickening and multilayering of basal lamina, degenerative and adaptative endothelial cell changes, alterations of pericytes and perivascular mononuclear cell infiltration. There was no correlationship between the intensity of the capillary abnormalities and the duration of the disease. In 12 patients with Raynaud's disease (RD) such changes were not found. Thus capillaries displayed normal-looking ultrastructural appearances. Mean capillary basal lamina width was consistently thicker in the PSS group than in the RD group (p less than 0.001). These observations are consistent with the hypothesis that the primary event in PSS occurs in the microvessels. It is suggested that labial salivary gland biopsy may be helpful for early recognition of PSS among subjects with Raynaud's syndrome.

摘要

通过电子显微镜检查发现,从20例进行性系统性硬化症(PSS)患者获取的唇唾液腺活检标本中存在毛细血管异常。这些异常包括基底膜明显增厚和多层化、内皮细胞的退行性和适应性改变、周细胞改变以及血管周围单核细胞浸润。毛细血管异常的严重程度与疾病持续时间之间没有相关性。在12例雷诺病(RD)患者中未发现此类变化。因此,毛细血管呈现出外观正常的超微结构。PSS组的平均毛细血管基底膜宽度始终比RD组厚(p<0.001)。这些观察结果与PSS的原发性病变发生在微血管中的假说一致。提示唇唾液腺活检可能有助于在雷诺综合征患者中早期识别PSS。

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