CAJ | 학술논문

目的：探讨原发性膜性肾病合并IgA肾病的临床、病理特点及诊断要点。方法分析我院确诊的2例膜性肾病合并IgA肾病病例的临床和病理资料，并进行文献复习。结果两例均为男性，年龄分别为17岁、73岁。例1表现为肾病综合征伴镜下血尿；例2以蛋白尿伴镜下血尿发病，9年后出现肾病综合征。两例血压及肾功能均正常。肾脏病理：光镜下均见肾小球基底膜空泡变性和增厚，系膜细胞和基质增生；免疫荧光见IgG和C3颗粒样沿肾小球毛细血管壁沉积，IgA团块状在系膜区沉积；电镜下均见肾小球上皮细胞下多数团块状电子致密物沉积，系膜区见团块状电子致密物沉积，上皮细胞足突广泛融合。两例均予激素治疗缓解，随访29～41个月肾功能正常。结论原发性膜性肾病合并IgA肾病临床表现无特异性，病理上兼具有膜性肾病和IgA肾病的病理特点。免疫荧光和电镜、免疫电镜在诊断中具有重要意义。
목적：탐토원발성막성신병합병IgA신병적림상、병리특점급진단요점。방법분석아원학진적2례막성신병합병IgA신병병례적림상화병리자료，병진행문헌복습。결과량례균위남성，년령분별위17세、73세。례1표현위신병종합정반경하혈뇨；례2이단백뇨반경하혈뇨발병，9년후출현신병종합정。량례혈압급신공능균정상。신장병리：광경하균견신소구기저막공포변성화증후，계막세포화기질증생；면역형광견IgG화C3과립양연신소구모세혈관벽침적，IgA단괴상재계막구침적；전경하균견신소구상피세포하다수단괴상전자치밀물침적，계막구견단괴상전자치밀물침적，상피세포족돌엄범융합。량례균여격소치료완해，수방29～41개월신공능정상。결론원발성막성신병합병IgA신병림상표현무특이성，병리상겸구유막성신병화IgA신병적병리특점。면역형광화전경、면역전경재진단중구유중요의의。
Objective To describe the clinical and pathological characteristics and diagnosis of membranous nephropathy combined with IgA nephropathy. Methods Two patients were confirmed to have idiopathic membranous nephropathy and IgA nephropathy in our hospital. We analyzed the clinical and pathological data and reviewed literature. Results Two patients were all male, 17 and 73 years old. One patient had nephrotic syndrome and microscopic hematuria, The other had proteinuria and microscopic hematuria initially, after 9 years performed nephrotic syndrome. Blood pressure and renal function of two patients were normal. The pathological characteristics included vacuolation and thickening of the glomerular basement membrane, mesangial cells and matrix hyperplasia. And IgA deposit in mesangium, IgG and C3 deposit in subepithelial site mainly. Lumpy electron dense deposited in subepithelial and mesangial area and foot processes fusion under electron microscopy. Two cases were remission after hormone therapy. During 29-41 months of follow up, the renal function remained normal. Conclusion Idiopathic membranous nephropathy combined with IgA nephropathy hadn't special clinical manifestation. Immunofluorescence, electron microscopy and immuno-electron microscopy in the diagnosis is important.