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[干燥综合征的肾脏受累——间质性肾炎和肾小球肾炎]

[Renal involvement in Sjögren's syndrome--interstitial nephritis and glomerulonephritis].

作者信息

Fujimoto T, Dohi K

机构信息

First Department of Internal Medicine, Nara Medical University.

出版信息

Nihon Rinsho. 1995 Oct;53(10):2495-502.

PMID:8531362
Abstract

Renal involvement is well recognized extraglandular manifestation of primary Sjögren's syndrome (SS). The most common histopathological lesion is an interstitial lymphocytic infiltrate with tubular atrophy and fibrosis. The clinical presentation may be hyposthenuria, overt or latent distal renal tubular acidosis and less commonly Fanconi's syndrome. These tubular dysfunctions correlate with the presence of interstitial lymphocytes. Immunoregulatory alterations consisting of impaired T-cell function and B-cell hyperactivity probably play a pathogenetic role in the development of interstitial nephritis in SS. Glomerulonephritis in SS has been described in a limited number of case reports. Variant modes of pathogenesis have been proposed in these cases where glomerulonephritis has been associated with immune complex deposition and cryoglobulinemia.

摘要

肾脏受累是原发性干燥综合征(SS)公认的腺外表现。最常见的组织病理学病变是间质淋巴细胞浸润伴肾小管萎缩和纤维化。临床表现可能为低渗尿、显性或隐性远端肾小管酸中毒,较少见的是范科尼综合征。这些肾小管功能障碍与间质淋巴细胞的存在相关。由T细胞功能受损和B细胞活性亢进组成的免疫调节改变可能在SS间质性肾炎的发生中起致病作用。SS中的肾小球肾炎仅在少数病例报告中有所描述。在这些病例中,已提出了不同的发病机制,其中肾小球肾炎与免疫复合物沉积和冷球蛋白血症有关。

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