膜性肾病合并IgA肾病患者临床及病理特征分析

Clinicopathologic analysis of membranous nephropathy combined with IgA nephropathy

目的 探讨膜性肾病合并IgA肾病(membranous nephropathy-IgA nephropathy,MN-IgAN)中PLA2R和THSD7A的表达及其与临床病理特征的关系。方法 收集108例经肾活检确诊的MN-IgAN患者的临床资料,另随机选取60例膜性肾病(membranous nephropathy,MN)及60例IgA肾病(IgA nephropathy,IgAN)作为对照;分析三组间的临床和病理特征。应用间接免疫荧光法检测MN-IgAN患者肾组织中PLA2R及THSD7A抗原表达。结果 与IgAN组相比,MN-IgAN组患者年龄高、BUN及血肌酐较低、肾病综合征比例高(P<0.05)。与MN组相比,MN-IgA组镜检血尿者多(P=0.002)、血清白蛋白高(P=0.016),其余临床资料相似。MN-IgA组与MN组电镜分期差异无统计学意义(P=0.472)。MN-IgAN组牛津分类E、S、T及C均比IgAN组低。MN-IgAN组PLA2R阳性率与MN组相比,差异无统计学意义(85.2%vs 86.7%,P=0.793),且以IgG4亚型为主。108例MN-IgAN中有2例THSD7A弱阳性,且这2例PLA2R均阳性。结论 MN-IgAN患者临床表现及电镜分期与MN组相似,牛津分类比IgAN低,MN-IgAN可能是原发性MN叠加病理类型较轻的IgAN的特殊亚型。

Purpose To investigate the relationship between clinicopathologic features and the expression of M-type phospholipase A2 receptor(PLA2 R) and thrombospondin type-1 domain-containing 7 A(THSD7 A) in of membranous nephropathy combined with IgA nephropathy(MN-IgAN).Methods 108 patients with biopsy-proved MN-IgAN were involved in this study.60 cases with membranous nephropathy and 60 cases with IgAN were randomly selected as controls.The clinical and pathological features of MN-IgAN group were compared with those of IgAN group and membranous nephropathy(MN) group.Indirect immunofluorescence was used to detect the expression of PLA2 R and THSD7 A in renal tissue of patients with MN-IgAN.Results Compared with IgAN,patients with MN-IgAN were older and had a lower BUN and serum creatinine,and a higher proportion of nephrotic syndrome(P<0.05).Compared with MN,patients with MN-IgAN had similar clinical features except for more microscopic hematuria(P=0.002),and higher serum albumin(P=0.016).There was no significant difference in electron microscope staging between MN-IgA group and MN group(P=0.472).Patients with MN-IgAN had lower Oxford Classification of E,S,T and C than IgAN.There was no significant difference in the positive of PLA2 R between MN-IgAN group and MN(85.2% vs 86.7%,P=0.793),the IgG4 subtype was dominant.THSD7 A was weakly positive in 2 of 108 patients with MN-IgAN and PLA2 R also positive in the 2 patients.Conclusion The clinical manifestations and electron microscope staging of MN-IgAN patients were similar to those of the MN group,the Oxford classification was lighter than that of IgAN.MN-IgAN may be a special pathological subtype of primary MN superimposed with a milder pathological type of IgAN.