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Patients With Combined Membranous Nephropathy and Focal Segmental Glomerulosclerosis Have Comparable Clinical and Autoantibody Profiles With Primary Membranous Nephropathy: A Retrospective Observational Study

机译:回顾性观察研究表明,合并膜性肾病和局灶性节段性肾小球硬化的患者具有与原发性膜性肾病相当的临床和自身抗体谱

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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 +/- 633.9pg/mol; P=0.002), but similar to the primary MN patients (267.9 +/- 147.5pg/mol). 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.
机译:据报道合并膜性肾病(MN)和局灶性节段性肾小球硬化(FSGS)的患者具有不同的临床意义。对于合并的肾小球病变的可能机制的研究是必要的,但很少。这项研究招募了20名同时患有MN和FSGS病变的患者。将65例原发性MN患者和56例原发性FSGS患者用作疾病对照。收集肾活检和随访期间的临床数据。检测了循环抗磷脂酶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)的水平。我们发现合并病变的患者在活检中表现出年龄更大,蛋白尿更少,白蛋白更高和肾功能更好。这些与原发性MN患者相当,但与原发性FSGS患者不同。与原发性MN和原发性FSGS患者相比,合并病灶的患者表现出更高的MN分期,FSGS分类无细胞变异,且更常见(100.0%)的肾小管间质损伤。在合并病变的患者中,有80.0%的患者具有循环抗PLA2R抗体,而68.4%的患者肾小球中以IgG4为主沉积,与原发性MN相当。合并病变的患者的尿suPAR浓度明显低于原发性FSGS患者(315.6 +/- 151.0 vs 752.1 +/- 633.9pg / mol; P = 0.002),但与原发性MN患者相似(267.9 +/-) 147.5pg / mol)。我们得出结论,合并了MN和FSGS的患者可能与原发性MN有相同的潜在发病机制。 FSGS病变可能是继发于原发性MN。

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