【摘要】 目的分析1型血小板反应蛋白7A域(THSD7A)相关特发性膜性肾病(IMN)的临床及病理特征。方法选择2017年7月～2019年10月武汉大学人民医院肾内科经肾穿刺活检确诊为IMN的患者110例。所有患者均行肾小球磷脂酶A2受体(PLA2R)抗原染色及血清THSD7A抗体及PLA2R抗体检测。据其结果将IMN分为THSD7A-IMN组、PLA2R-IMN组、阴性组3组,分析比较3组的临床病理特征。进一步将THSD7A-IMN组分为单抗体阳性组及双抗体阳性组,比较二者临床病理特征差异。结果在110例IMN患者中,THSD7A抗体阳性患者21例(19.1%),包括单抗体阳性5例(4.5%),双抗体阳性16例(14.5%)。THSD7A-IMN主要存在于男性患者(男性∶女性为14∶7)中,平均年龄为54.3±12.3岁,平均白蛋白为26.0±7.6g/L,平均尿蛋白量为7.9±4.4g/24h,其血清补体C4水平显著低于PLA2R-IMN及阴性组,差异有统计学意义(P均<0.01)。THSD7A-IMN组及PLA2R-IMN组的尿蛋白量显著高于阴性组(P分别为0.043、0.044),而两组的白蛋白水平显著低于阴性组,差异均有统计学意义(P分别为0.010、0.015)。血清THSD7A抗体水平与尿蛋白量不相关(r=0.135,P=0.561)。21例THSD7A-IMN中,单抗体阳性组与双抗体阳性组临床病理特征比较,差异无统计学意义(P均> 0.05)。结论血清THSD7A抗体阳性的IMN流行率不低,其临床病理特点与PLA2R-IMN相似。THSD7A也可表达于PLA2R阳性的IMN患者中,且其临床病理特点不因单、双抗体阳性而有所差异。更多还原

【Abstract】 Objective To investigate the clinical and pathological charac teristics of thrombospondin type-1 domain-containing7 A( THSD7A) related id iopathic membranous nephropathy( IMN). Methods One hundred and ten patients with biopsy-proven IMN at the Renmin Hospital of Wuhan University from July 2017 to October 2019 were enrolled. All patients were tested with glomerular phospholipase A2 receptor( PLA2R) antigen staining and serum THSD7A-Ab and PLA2R-Ab. The 110 patients with IMN patients were divided into THSD7A-IMN,PLA2R-IMN and negative groups to retrospectively analyze their clinicopa thological characteristics. Further,the THSD7A-IMN group was divided into the single-Ab positive group and the double-Ab positive group to analyze the differences of their clinicopathological characteristics. Results A total of 21 cases of THSD7A-IMN were enrolled,among which 5( 4. 5%) cases were single-Ab positive and 16( 14. 5%) cases were double-Ab positive. The patients of THSD7A-IMN were most often male( male-to-female ratio of 2. 0) with a mean age of 54. 3 ± 12. 3 years,a mean albumin of 26. 0 ± 7. 6 g/L and a mean proteinuria of 7. 9 ± 4. 4 g per day,and theirs serum complement C4 level was lower than that of the PLA2R-IMN and negative group with statistically significance( P <0. 01). Compared with individuals of negative group,patients in THSD7A-IMN and PLA2R-IMN groups had higher urine protein excretion( P = 0. 043,P = 0. 044) and lower albumin( P = 0. 010,P = 0. 015) all with statistically significance. There was no correlation between serum THSD7A-Ab level and urine protein excretion( r = 0. 135,P = 0. 561). Among the 21 case of THSD7A-IMN,the differences of clinicopatho logical characteristics between single-Ab positive group and double-Ab positive group were not statistically signific ant. Conclusion The prevalence of serum THSD7A-Ab positive IMN is not low,and its clinicopathological characteristics are similar to those of PLA2R-IMN. THSD7A can also be expressed in IMN patients with positive PLA2R,and its clinicopathological characteristics are not different due to single or double antibody positive.更多还原