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目的:分析银屑病相关性膜性肾病的临床表现、免疫学和肾脏病理特点。方法:回顾性分析解放军肾脏病研究所1998年2月至2008年6月行肾活检术诊断为银屑病相关性膜性肾病共17例患者,分析其临床表现、实验室指标、肾活检病理及免疫病理特点。应用免疫组化方法观察肾小球中IgG亚型的分布。结果:(1)男性青壮年患者多见,男性12例,女性5例。平均年龄(41.7±13.5)岁。(2)17例患者均为寻常型银屑病。银屑病史为1～20年,平均8.1年。均先以银屑病起病,肾脏损害在银屑病发病后(86.1±62.2)月(10～179.5月)被发现。肾脏损害的平均病程为10.6月(0.5～48月)。多数患者临床表现为典型的肾病综合征,尿蛋白平均(3.94±2.29)g/24h(0.59～8.71g/24h)。均无发作性肉眼血尿,仅2例存在少量镜下血尿。(3)肾小管功能明显受损,表现为尿NAG酶升高12例(71%),RBP升高8例(47%)。尿渗透压低于正常值14例(82%)。(4)肾组织光镜下多见节段系膜增生性病变,肾小球内皮细胞肿胀、成对,8例(47%)存在肾小球内中性粒细胞和单核细胞浸润。9例(53%)肾间质内可见小灶性单核细胞浸润。6例(35%)间质轻度纤维化。10例(59%)见数处间质小动脉透明变性,弹力层分层,1例(6%)可见血管壁炎细胞浸润。(5)免疫病理检查示5例患者同时伴随C4和C1q沉积。2例可见IgG、IgA、IgM、C3、C4、C1q均沉积。(6)肾组织IgG亚型染色肾小球IgG1、IgG4均有沉积,IgG1相对更强,无IgG3沉积。3例患者可见IgG亚型染色节段分布。另2例患者可见小管基膜、管周毛细血管IgG1和IgG4沉积。结论:银屑病相关性膜性肾病多见于男性青壮年。临床主要表现为肾病综合征,无明显镜下血尿。肾小管功能明显受损。肾组织光镜下多见节段系膜增生性病变,肾小球内皮细胞肿胀、成对,浸润细胞多见,肾间质小动脉血管壁也可见炎性细胞浸润。免疫荧光染色可见部分患者C4和C1q共同沉积。
Objective: To analyze the clinical manifestations, immunology and pathological features of psoriasis-associated membranous nephropathy. Methods: A retrospective analysis of the PLA Renal Disease Institute from February 1998 to June 2008 renal biopsy diagnosis of psoriasis associated with membranous nephropathy in 17 patients, analysis of its clinical manifestations, laboratory parameters, renal biopsy pathology And immunopathological features. Immunohistochemistry was used to observe the distribution of IgG subtypes in glomeruli. Results: (1) Male patients were more common in young adults, 12 males and 5 females. The average age (41.7 ± 13.5) years old. (2) 17 patients were all psoriasis vulgaris. Psoriasis history of 1 to 20 years, an average of 8.1 years. All started with psoriasis, kidney damage after the onset of psoriasis (86.1 ± 62.2) months (10 ~ 179.5 months) was found. The average duration of renal damage was 10.6 months (0.5 ~ 48 months). The clinical manifestations of most patients with typical nephrotic syndrome, urinary protein average (3.94 ± 2.29) g / 24h (0.59 ~ 8.71g / 24h). No episodes of gross hematuria, only 2 cases of microscopic hematuria. (3) Renal tubular function was significantly impaired, manifested by increased urinary NAG enzyme in 12 (71%) and elevated RBP in 8 (47%). Urinary osmolality was lower than normal in 14 cases (82%). (4) Renal tissue under the light microscope more common mesangial proliferative lesions, glomerular endothelial cell swelling, paired, 8 cases (47%) glomerular neutrophil and mononuclear cell infiltration. Nine cases (53%) of renal interstitial infiltration of small focal can be seen. Six cases (35%) interstitial fibrosis. 10 cases (59%) see the number of clear degeneration of the interstitial arterioles, stratified elastic layer, 1 case (6%) visible infiltration of vascular parietal cells. (5) Immunopathological examination showed that 5 patients had both C4 and C1q deposition. 2 cases of visible IgG, IgA, IgM, C3, C4, C1q are deposited. (6) Kidney IgG subtype stain glomerular IgG1, IgG4 are deposited, IgG1 is relatively stronger, no IgG3 deposition. Three patients showed IgG subtype staining segment distribution. The other two patients showed tubule basement membrane, tube capillaries IgG1 and IgG4 deposition. Conclusion: Psoriasis-associated membranous nephropathy is more common in young males. The main clinical manifestations of nephrotic syndrome, no significant microscopic hematuria. Tubular function was significantly impaired. Under the light microscope of renal tissue, the most common segments of mesangial proliferative lesions, glomerular endothelial cell swelling, paired, infiltrating cells more common, renal interstitial arterial wall can also be seen inflammatory cell infiltration. Immunofluorescent staining showed that some patients co-deposited with C4 and C1q.