Fuller T J, Richman A V, Auerbach D, Alexander R W, Lottenberg R, Longley S
Am J Med. 1977 May;62(5):761-4. doi: 10.1016/0002-9343(77)90880-4.
Renal involvement and hypocomplementemia in mixed connective tissue disease are reported to be rare. A patient is described here with mixed connective tissue disease and persistently low serum C'3 levels in whom renal insufficiency and nephrotic syndrome developed secondary to immune-complex glomerulonephritis. Light microscopy of the renal biopsy specimen showed predominantly a membranous lesion. Immunofluorescent staining showed granular deposition along the basement membrane of immunoglobulin G, immunoglobulin M, fibrinogen and C3. Electron microscopy showed numerous electron-dense deposits along the glomerular capillary membrane and in the mesangium.
据报道,混合性结缔组织病中的肾脏受累和低补体血症较为罕见。本文描述了一名患有混合性结缔组织病且血清C'3水平持续降低的患者,该患者继发于免疫复合物性肾小球肾炎而出现肾功能不全和肾病综合征。肾活检标本的光镜检查主要显示为膜性病变。免疫荧光染色显示免疫球蛋白G、免疫球蛋白M、纤维蛋白原和C3沿基底膜呈颗粒状沉积。电镜检查显示沿肾小球毛细血管膜和系膜中有大量电子致密沉积物。