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Seropositive PLA2R-associated membranous nephropathy but biopsy-negative PLA2R staining

机译:血清阳性 PLA2R 相关膜性肾病但活检 PLA2R 染色阴性

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Background. Serum phospholipase A2 receptor (PLA2R) anti-body (SAb) and glomerular deposits of PLA2R antigen (GAg) have been tested widely in idiopathic membranous nephropathy (MN). Recently, we noticed a special form of PLA2R-associated MN with positive circulating PLA2R antibody but negative PLA2R deposits in the glomeruli by immunofluorescence on frozen tissue (IF-F). The significance of this form of PLA2R-associated MN is yet to be elucidated. This study aimed to explore the clinicopathological features of these PLA2R-associated MN patients. Methods. This study enrolled 229 biopsy-proven PLA2R-associated MN patients with SAb+. SAb was measured by enzyme-linked immunosorbent assay, and GAg was detected by IF-F. These patients were divided into SAb+/GAg+ and SAb+/GAg- groups. Clinicopathological characteristics of SAb+/GAg+ and SAb+/GAg- PLA2R-associated MN patients were compared. PLA2R antigens of 19 SAb+/GAg- PLA2R-associated MN patients were verified by immunohistochemistry on paraffin tissue (IHC-P). Results. Among 229 SAb+ PLA2R-associated MN patients, 210 (91.70) were GAg+ and 19 (8.3) were GAg-. These 19 SAb+/GAg- PLA2R-associated MN patients presented positive PLA2R deposits by IHC-P. Compared with SAb+/GAg+ PLA2R-associated MN patients, SAb+/GAg- PLA2R-associated MN patients had higher levels of serum PLA2R anti-body (P = 0.004), increased proteinuria (P = 0.008), lower serum albumin (P = 0.019), more prominent chronic pathological lesions in terms of glomerulosclerosis score (P = 0.025), interstitial fibrosis score (P = 0.016), tubular atrophy score (P = 0.010) and total renal chronicity score (P = 0.010), and were more likely to be accompanied by focal segmental glomerulosclerosis (P = 0.014). Higher SAb level was associated with the total renal chronicity score (odds ratio per 100 RU/mL, 1.16; 95 confidence interval 1.01-1.33; P = 0.033). Conclusions. PLA2R-associated MN patients with seropositive PLA2R antibody but negative PLA2R deposits in the glomeruli by IF-F have higher levels of SAb and worse clinicopathological manifestations compared with their double-positive counterparts. IHC-P can be an alternative technique to reveal PLA2R glomerular deposits.
机译:背景。血清磷脂酶 A2 受体 (PLA2R) 抗体 (SAb) 和肾小球沉积的 PLA2R 抗原 (GAg) 已在特发性膜性肾病 (MN) 中广泛检测。最近,我们注意到一种特殊形式的 PLA2R 相关 MN,循环 PLA2R 抗体呈阳性,但通过冷冻组织免疫荧光 (IF-F) 在肾小球中沉积 PLA2R 呈阴性。这种形式的PLA2R相关膜性肾病的意义尚未阐明。本研究旨在探讨这些PLA2R相关膜性肾病患者的临床病理特征。方法。该研究招募了 229 例经活检证实的 PLA2R 相关 SAb+ 膜性肾病患者。酶联免疫吸附法测定SAb,IF-F法检测GAg。将这些患者分为SAb+/GAg+组和SAb+/GAg-组。比较SAb+/GAg+和SAb+/GAg-PLA2R相关膜性肾病患者的临床病理特征。通过石蜡组织免疫组化(IHC-P)验证了19例SAb+/GAg-PLA2R相关膜性肾病患者的PLA2R抗原。结果。229例SAb+、PLA2R相关膜性肾病患者中,GAg+210例(91.70%),GAg-19例(8.3%)。这 19 例 SAb+/GAg-PLA2R 相关 MN 患者通过 IHC-P 呈现阳性 PLA2R 沉积。与SAb+/GAg+ PLA2R相关膜性肾病患者相比,SAb+/GAg-PLA2R相关膜性肾病患者血清PLA2R抗体水平较高(P=0.004),蛋白尿升高(P=0.008),血清白蛋白降低(P=0.019),肾小球硬化评分(P=0.025)、间质纤维化评分(P=0.016)、肾小管萎缩评分(P=0.010)和总肾慢性病评分(P=0.010)等慢性病理病变更为突出,更可能伴有局灶节段性肾小球硬化(P=0.014)。较高的SAb水平与总肾脏慢性评分相关(比值比/100 RU/mL,1.16;95%置信区间1.01-1.33;P = 0.033)。结论。PLA2R相关膜性肾病患者血清学PLA2R抗体阳性,但IF-F在肾小球中PLA2R沉积阴性,与双阳性患者相比,SAb水平更高,临床病理表现更差。IHC-P 可以作为揭示 PLA2R 肾小球沉积物的替代技术。

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