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IgA/IgG冷球蛋白血症中的快速进展性肾小球肾炎

Rapidly progressive glomerulonephritis in IgA/IgG cryoglobulinemia.

作者信息

Weber M, Köhler H, Fries J, Thoenes W, Meyer zum Büschenfelde K H

出版信息

Nephron. 1985;41(3):258-61. doi: 10.1159/000183593.

Abstract

Mixed IgA/IgG cryoglobulins were found in the serum of a 48-year-old man suffering from rapidly progressive glomerulonephritis (RPGN) with crescent formation. The type-II cryoglobulins were composed of monoclonal IgA1-kappa and polyclonal IgG, with the IgA possessing antibody activity against the IgG. The RPGN was of the immune complex type with granular deposits of IgA, IgG, and C3 on immunofluorescence microscopy and preponderant subendothelial deposits on electron microscopy. Occluding protein thrombi could be demonstrated in several glomerular capillary loops. Removal of the cryoglobulins from the patient's serum by plasmapheresis and immunosuppression was paralleled by a remarkable improvement in renal function with fall of serum creatinine values from 13.6 mg/dl (1,202.2 mumol/l) to 2.8 mg/dl (247.5 mumol/l), a resolution of the glomerular lesions, and clinical improvement as well. Our observations suggest that the crescentic glomerulonephritis may be due to an immune complex-like deposition of the cryoproteins. We conclude that crescentic glomerulonephritis in IgA/IgG cryoglobulinemia has to be considered as an autoimmune form of RPGN.

摘要

在一名患有快速进展性肾小球肾炎(RPGN)伴新月体形成的48岁男性患者血清中发现了混合性IgA/IgG冷球蛋白。II型冷球蛋白由单克隆IgA1-κ和多克隆IgG组成,其中IgA具有针对IgG的抗体活性。该RPGN属于免疫复合物型,免疫荧光显微镜检查显示IgA、IgG和C3呈颗粒状沉积,电子显微镜检查显示主要为内皮下沉积。在几个肾小球毛细血管袢中可证实存在阻塞性蛋白血栓。通过血浆置换和免疫抑制从患者血清中去除冷球蛋白后,肾功能显著改善,血清肌酐值从13.6mg/dl(1202.2μmol/l)降至2.8mg/dl(247.5μmol/l),肾小球病变消退,临床症状也有所改善。我们的观察结果表明,新月体性肾小球肾炎可能是由于冷球蛋白的免疫复合物样沉积所致。我们得出结论,IgA/IgG冷球蛋白血症中的新月体性肾小球肾炎应被视为RPGN的一种自身免疫形式。

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