Patients With Combined Membranous Nephropathy and Focal Segmental Glomerulosclerosis Have Comparable Clinical and Autoantibody Profiles With Primary Membranous Nephropathy: A Retrospective Observational Study.
Patients With Combined Membranous Nephropathy and Focal Segmental Glomerulosclerosis Have Comparable Clinical and Autoantibody Profiles With Primary Membranous Nephropathy: A Retrospective Observational Study.
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合并膜性肾病和局灶节段性肾小球硬化患者的临床和自身抗体特征与原发性膜性肾病相似：一项回顾性观察研究
DOI：
10.1097/md.0000000000003786
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发表时间：
2016-05
期刊：
影响因子：
1.6
通讯作者：
Zhao MH
中科院分区：
文献类型：
--
作者：
Gu QH;Cui Z;Huang J;Zhang YM;Qu Z;Wang F;Wang X;Wang SX;Liu G;Zhao MH
膜性肾病(MN)合并局灶性节段性肾小球硬化(FSGS)的临床意义各不相同。对合并肾小球损害的可能机制的研究是必要的，但很少。20名同时患有MN和FSGS皮损的患者参加了研究。65例原发MN患者和56例原发FSGS患者作为疾病对照。收集肾活检和随访期间的临床资料。检测循环抗磷脂酶A2受体(PLA2R)抗体、肾小球PLA2R表达、IgG4沉积和可溶性尿激酶受体(SuPAR)水平。我们发现，合并病变的患者在活检中表现为年龄较大、蛋白尿较少、白蛋白较高、肾功能较好。这些指标与原发MN患者相似，但与原发FSGS患者不同。与原发MN和原发FSGS患者相比，合并病变的患者表现出更高的MN分期，FSGS分类中无细胞变异，以及更常见的肾小管间质损伤(100.0%)。在合并病变的患者中，80.0%的患者循环中有抗PLA2R抗体，68.4%的患者肾小球以IgG4为主，与原发MN相似。合并皮损的患者尿suPAR浓度显著低于原发肾小球肾炎患者(315.6 ± 151.0 vs 752.1 ± 633.9 pg/μ；P = 0.002)，但与原发肾小球肾炎患者(267.9 ± 147.5 pg/μm ol)相似。我们的结论是，合并MN和FSGS的患者可能与原发MN具有相同的潜在发病机制。FSGS病变可能是原发MN的继发性病变。
Patients with combined membranous nephropathy (MN) and focal segmental glomerulosclerosis (FSGS) have been reported with different clinical significance. Investigations on the possible mechanisms of the combined glomerular lesions are necessary but scarce. Twenty patients with both MN and FSGS lesions were enrolled in the study. Sixty-five patients with primary MN and 56 patients with primary FSGS were used as disease controls. Clinical data on renal biopsy and during follow-up were collected. Circulating anti-phospholipase A2 receptor (PLA2R) antibody, glomerular PLA2R expression, IgG4 deposition, and soluble urokinase receptor (suPAR) levels were detected. We found that patients with combined lesions presented with older age, less proteinuria, higher albumin, and better renal function on biopsy. These were comparable to the patients with primary MN, but differed from the patients with primary FSGS. Patients with combined lesions showed higher stages of MN, no cellular variant on FSGS classification, and more common (100.0%) tubulointerstitial injury than both primary MN and primary FSGS patients. In the patients with combined lesions, 80.0% had circulating anti-PLA2R antibody and 68.4% had IgG4 predominant deposition in glomeruli, which were comparable to primary MN. The patients with combined lesions had significantly lower urinary suPAR concentrations, than the primary FSGS patients (315.6 ± 151.0 vs 752.1 ± 633.9 pg/μmol; P = 0.002), but similar to the primary MN patients (267.9 ± 147.5 pg/μmol). We conclude that patients with combined MN and FSGS may share the same underlying pathogenesis with primary MN. The FSGS lesion might be secondary to primary MN.