Kurihara I, Saito T, Sato H, Chiba J, Saito J, Soma J, Ito S
Second Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan.
Am J Kidney Dis. 1998 Jul;32(1):E4. doi: 10.1053/ajkd.1998.v32.pm10074587.
We report a case of immunotactoid glomerulopathy with severe hypocomplementemia. The patient was a 47-year-old woman who presented with pitting edema, proteinuria, and hypertension. Serological testings were negative or within normal limits except for hypocomplementemia. There were no findings of hematopoietic diseases, cryoglobulinemia, and systemic lupus erythematosus. The renal biopsy specimen showed membranoproliferative glomerulonephritis with numerous periodic acid-Schiff (PAS)-positive deposits. Under electron microscopy, however, microtubular structure was shown in the mesangial matrix and the subendothelial and subepithelial spaces of the peripheral capillary loops. These histological features were compatible with those of immunotactoid glomerulopathy. Although conventional oral steroid therapy failed to have an effect on proteinuria and hypocomplementemia over 3 months, steroid pulse therapy brought dramatic relief: complete remission of proteinuria and normalization of hypocomplementemia. These findings suggest that intensive immunosuppressive therapy may cure a kind of immunotactoid glomerulopathy with hypocomplementemia.
我们报告一例伴有严重低补体血症的免疫触须样肾小球病。患者为一名47岁女性，表现为凹陷性水肿、蛋白尿和高血压。除低补体血症外，血清学检查均为阴性或在正常范围内。未发现造血系统疾病、冷球蛋白血症和系统性红斑狼疮。肾活检标本显示为膜增生性肾小球肾炎，伴有大量过碘酸-希夫（PAS）阳性沉积物。然而，在电子显微镜下，系膜基质以及外周毛细血管袢的内皮下和上皮下间隙可见微管结构。这些组织学特征与免疫触须样肾小球病相符。尽管传统口服类固醇治疗3个月以上对蛋白尿和低补体血症均无效果，但类固醇冲击疗法带来了显著缓解：蛋白尿完全缓解，低补体血症恢复正常。这些发现提示，强化免疫抑制治疗可能治愈一种伴有低补体血症的免疫触须样肾小球病。
