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目的:比较系膜增生狼疮性肾炎伴足细胞病与不伴足细胞病患者的临床病理特征及预后差别。方法:108例肾活检显示肾小球系膜增生性狼疮性肾炎患者[女99例,男9例,中位年龄30(21~39.3)岁],根据电镜检查足突融合和蛋白尿程度分为足细胞病组(足突融合范围>50%且伴肾病综合征)和系膜增生组(足突融合≤50%)。系膜增生和肾小管间质病变程度进行半定量评分,系膜增生组根据有无蛋白尿分为血尿组(仅有镜下血尿,无蛋白尿)和蛋白尿组(蛋白尿伴或不伴血尿)两个亚组。比较不同组别临床病理特征、治疗反应及预后的差异。结果:足细胞病组和系膜增生组分别有28例和80例,两组足突融合比例分别为82.5%(70%,85%)和15%(10%,25%)。足细胞病组以肾病首发(60.7%vs 23.8%,P<0.001)、肾病综合征(100%vs1.25%,P<0.001)、急性肾损伤(25.0%vs 2.5%,P=0.001)比例及肾小管间质急性病变程度均显著高于系膜增生组,而关节炎、发热、低补体C4血症(28.6%vs 52.5%,P=0.029)、抗磷脂抗体阳性及血尿发生率(21.4%vs42.5%,P=0.047)显著低于系膜增生组。两组系膜区免疫球蛋白和补体沉积、系膜增生程度无明显差异,足细胞病组肾小管免疫球蛋白沉积率显著高于系膜增生组(35.7%vs 2.5%,P<0.001)。激素或激素联合免疫抑制剂治疗两组缓解率无明显差异,足细胞病组的肾脏复发率明显高于系膜增生组(53.6%vs 29.0%,P=0.025)。在分别随访74个月(足细胞病组)和51个月(系膜增生组)期间,两组均无死亡或进展为ESRD。结论:有足细胞病的系膜增生性狼疮性肾炎的临床和形态学特征及转归与无足细胞病的系膜增生性狼疮性肾炎存在显著差异,两者应属于不同类型的狼疮性肾炎。
Objective: To compare the clinicopathological characteristics and prognosis of patients with mesangial proliferative lupus nephritis with or without podocytosis. Methods: One hundred and eighty patients with renal mesangial proliferative lupus nephritis (99 women, 9 males, median age 30 (21-39.3) years) were examined by renal biopsy. According to electron microscopy, For the podocyte group (foot process fusion range> 50% with nephrotic syndrome) and mesangial proliferation group (foot process fusion ≤ 50%). Mesangial hyperplasia and tubulointerstitial lesions were semi-quantitative score, mesangial proliferative group according to the presence of proteinuria is divided into hematuria group (only microscopic hematuria, proteinuria) and proteinuria group (proteinuria with or without Hematuria) two subgroups. The differences of clinicopathological characteristics, treatment response and prognosis of different groups were compared. Results: There were 28 cases and 80 cases in podocyte group and mesangial proliferation group respectively. The fusion rates of the two groups were 82.5% (70%, 85%) and 15% (10%, 25%) respectively. The group of patients with podocytic disease showed the highest incidence of nephropathy (60.7% vs 23.8%, P <0.001), nephrotic syndrome (100% vs 1.25%, P <0.001) and acute renal injury (25.0% vs 2.5%, P = 0.001) And tubulointerstitial lesions were significantly higher than the mesangial proliferation group, and arthritis, fever, hypoattenuated C4 hyperlipidemia (28.6% vs 52.5%, P = 0.029), antiphospholipid antibodies and hematuria (21.4 % vs42.5%, P = 0.047) was significantly lower than mesangial proliferation group. There was no significant difference in immunoglobulin, complement deposition and mesangial hyperplasia between the two mesangial regions. The deposition rate of tubular immunoglobulin in podocyte group was significantly higher than that in mesangial proliferation group (35.7% vs 2.5%, P <0.001). Hormone or hormone combination immunosuppressive therapy had no significant difference in remission rate between the two groups. The rate of renal recurrence in podocyte group was significantly higher than that in mesangial proliferation group (53.6% vs 29.0%, P = 0.025). During the 74-month follow-up period (podocyte group) and 51 months (mesangial proliferation group), neither group died nor progressed to ESRD. Conclusions: The clinical and morphological features of mesangial proliferative lupus nephritis with podocytic disease are significantly different from that of mesangial proliferative lupus nephritis without podocytic disease. Both of them should belong to different types of lupus nephritis .