Shouno Y, Shimokama T, Sakemi T, Harada A, Watanabe T
Department of Pathology, Saga Medical School, Japan.
Nihon Jinzo Gakkai Shi. 1993 Sep;35(9):1043-50.
Using 100 serial sections for light microscopy, we studied the clinicopathologic characteristics of IgA nephropathy with severe proteinuria. Eight out of 128 cases with IgA nephropathy exhibited severe proteinuria of 3.0g/day or more. These cases consisted of 5 males and 3 females with an age range from 15 to 73 years. Daily proteinuria ranged from 3.3 to 7.1 g. At the time of biopsy, the serum creatinine level was 1.2 mg/dl or more in 6 cases; two of these developed end-stage renal failure during the follow-up period. In most of the cases, routine sections for light microscopy revealed moderate to severe mesangial proliferation and tubulointerstitial damage. In 7 cases, variable percentages of glomeruli, ranging from 20% to 95%, were involved by chronic lesion represented by sclerosis and fibrous crescents/adhesion, while only 2 cases exhibited active lesion characterized by segmental glomerular necrosis and/or cellular/fibrocellular crescents. In contrast, thorough observation of 100 additional serial sections disclosed the focal presence of active lesion in all except one case with features of endstage kidney. In addition, the highest percentage of glomeruli with active lesion in the serial sections correlated well with the severity of proteinuria. The results suggested that severe proteinuria in IgA nephropathy depends on, at least in part, focal outbreak of active lesion characterized by segmental glomerular necrosis and cellular/fibrocellular crescents.
我们使用100个连续切片进行光学显微镜检查,研究了重度蛋白尿型IgA肾病的临床病理特征。128例IgA肾病患者中有8例出现了每天3.0克及以上的重度蛋白尿。这些病例包括5名男性和3名女性,年龄在15至73岁之间。每日蛋白尿范围为3.3至7.1克。活检时,6例患者的血清肌酐水平为1.2毫克/分升或更高;其中2例在随访期间发展为终末期肾衰竭。在大多数病例中,光学显微镜常规切片显示中度至重度系膜增生和肾小管间质损伤。7例中,20%至95%不等的肾小球出现了以硬化和纤维性新月体/粘连为代表的慢性病变,而只有2例表现为以节段性肾小球坏死和/或细胞性/纤维细胞性新月体为特征的活动性病变。相比之下,对另外100个连续切片的详细观察发现,除1例具有终末期肾病特征的病例外,所有病例均有局灶性活动性病变。此外,连续切片中活动性病变肾小球的最高比例与蛋白尿的严重程度密切相关。结果表明,IgA肾病中的重度蛋白尿至少部分取决于以节段性肾小球坏死和细胞性/纤维细胞性新月体为特征的局灶性活动性病变的爆发。