摘要
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 investigate the clinical and pathological features in patients of membranous nephropathy （MN） associated with psoriasis vulgaris. Methodology：Seventeen patients of MN associated with psoriasis vulgaris were included in this retrospective study. The renal biopsy was performed in all patients. Immunohistochemical method was used to detect the distribution of IgG subclasses in renal tissues. Results：①They were 12 males and 5 females with the mean age of （41.7±13.5） years old. ②The mean history of psoriasis vulgaris was 8.1 years, and the time between psoriasis and nephropathy were from 10 to 179.5 months （ mean of 86.1±62.2）. 16 cases presented edema. The average of urinary protein excretion was （3.94±2.29） g/24 h. ③Elevated urinary NAG （ 12 cases, 71% ） and RBP （8 cases, 47% ）, decreased urinary osmotic pressure （ 14 cases, 82% ） were noted. ④The histological examination showed thickening of glomerular capillary walls and mild mesangial proliferation. Endothelial cell proliferation and leukocyte infiltration were observed in 8 cases. Vascular involvement, including arteriolar hyalinosis and incrassation, was observed in 10 patients. ⑤By immunofluorescent microscopy, the granular deposits of IgG, C3, C4 and Clq along the capillary walls were in 5 patients, and the granular deposits of IgG, IgA, IgM, C3, CA and Clq along the capillary walls were in 2 cases. ⑥The deposition of IgG1 and IgG4 was also detected in the glomerular capillary walls. The deposition of IgG1 was stronger than IgG4. The segmental deposition of IgG subclasses was found in 3 patients. In addition to, the deposition of IgG subclasses on the peritubular capillary and the tubular basement membrane was in 2 cases. Conclusion：The patients of MN associated with psoriasis vulgaris presented edema and nephropathic proteinuria. The histological examination showed non-typical membranous nephropathy with obvious tubulointerstitial lesions. The granular deposits of C4 and C1q were observed in some patients by immunohistochemical examination. The deposition of IgG1 was stronger than IgG4.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
2008年第3期229-235,241,共8页
Chinese Journal of Nephrology,Dialysis & Transplantation
关键词
银屑病
膜性肾病
临床
病理
psoriasis
membranous nephropathy
clinical
pathological
