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疱疹样皮炎中肾小球基底膜的IgA假线性沉积。

IgA pseudo-linear deposits in glomerular basement membranes in dermatitis herpetiformis.

作者信息

Bartoli E, Bosincu L, Costanzi G, Denti S, Olmeo N A, Soggia G

出版信息

Am J Clin Pathol. 1982 Sep;78(3):377-80. doi: 10.1093/ajcp/78.3.377.

Abstract

An 18-year-old-woman, with dermatitis herpetiformis, acute glomerulonephritis, malabsorption and villous atrophy due to massive infiltration of IgA producing plasma cells was studied. By light microscopy, her renal biopsy specimen showed heavy immunofluorescence for IgA, a mixed proliferative and membranous lesion with occasional crescents and focal sclerosis. Electron microscopic examination revealed three main lesions: (1) swelling and bleb formation in endothelial cells, (2) extensive fusion of foot processes of podocytes, and (3) a dense quasi-linear, continuous deposition of immune complexes, encompassing several loops; they were characteristically located within the basement membrane at the boundary between the lamina densa and the lamina rara interna. At occasional points where the immune complexes had reached the outer aspects of the basement membrane, there was damage to the podocyte cytoplasm. This electron microscopic aspect supports the interpretation that these immune complexes, although non-complement fixing, exhibit a high damaging potential, leading to relentless disease progression.

摘要

对一名18岁患有疱疹样皮炎、急性肾小球肾炎、吸收不良以及因产生IgA的浆细胞大量浸润导致绒毛萎缩的女性患者进行了研究。光镜下,她的肾活检标本显示IgA有强烈免疫荧光,为混合性增生性和膜性病变,偶见新月体和局灶性硬化。电子显微镜检查发现三个主要病变:(1)内皮细胞肿胀和形成泡状突起;(2)足细胞足突广泛融合;(3)免疫复合物呈致密的准线性连续沉积,环绕多个袢环;其特征性地位于致密层和内疏松层之间的基底膜内。在免疫复合物偶尔到达基底膜外侧的部位,足细胞胞质有损伤。这种电子显微镜表现支持这样的解释,即这些免疫复合物虽然不固定补体,但具有很高的破坏潜能,并导致疾病持续进展。

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