65例伴有慢性肾功能不全的IgA肾病临床病理分析

The clinical and pathological analysis of 65 cases IgA nephropathy with chronic renal failure

目的探讨IgA肾病伴慢性肾功能不全的临床病理特点。方法回顾性分析65例伴慢性肾功能不全IgA肾病患者的临床病理资料，所有病例根据估算的肾小球滤过率（eGFR）水平分为A、B两组；分别按WHO分型、牛津病理分级（MSET）进行病理分型；Katafuchi R半定量积分法评估。肾脏病理损害程度并分析其与eGFR水平的相关性。结果65例伴慢性肾功能不全的IgA肾病按WHO分型和牛津病理分级，最常见的病理类型分别是增生硬化型（PSGN）和M1S1EOT0，其中绝大数患者出现肾小球硬化；免疫荧光检查以单纯IgA沉积最多见（46．1％），44．6％的患者伴c3沉积，4．6％伴C1q沉积；相关性分析显示eGFR水平与肾小管萎缩／间质纤维化（T）及肾小球球性硬化率呈负相关（P〈0．05），而与系膜细胞增生、节段硬化无明显相关（P〉0．05）。结论IgA肾病伴慢性肾功能不全好发于中青年，临床情况差，病理损害重，肾功能情况与肾小管萎缩／间质纤维化程度及肾小球球性硬化率有关。

Objective To investigative clinicopathlogical characteristics of IgA nephropathy with chronic renal failure. Methods Clinical and pathologic materials from 65 cases of IgA nephropathy with chronic renal failure were reviewed. All cases were divided into two groups according to their eGFR levels. Pathlogical characteristics of all the cases were analyzed according to WHO and Oxford Classification. The severity of pathological lesions was evaluated by Katafuchi R semiquantitative scoring system, and their relationship with level of eGFR was analyzed. Results In all 65 cases, the most common pathological type were proliferative sclerosing glomerulonephritis（PSGN） and MIS1EOT0 according to WHO and Oxford Classification respectively and most of the cases had glomerulosclerosis. Simple IgA deposition was the most common immunopathologic type. Of all the cases,44.6% accompanied with C3 while 4.6% with Clq. Further analysis revealed that level of eGFR was negatively related to the degree of renal interstitial fibrosis,renal atrophy and global sclerosis（ P 〈 0. 05 ）, but eGFR had no relationship with degree of mesangial cell proliferation and segmental glomerulosclerosis （P 〉 0. 05 ）. Conclusion IgA nephropathy with chronic renal failure usually occurred in young adults, and it had severe clinical condition and pathological changes,and there were some relationship between renal function and degree of renal interstitial fibrosis, renal atrophy and global sclerosis.