老年抗中性粒细胞胞质抗体相关性血管炎合并肾脏免疫复合物沉积的临床与病理特点分析

Clinical and Pathological Characteristics of ANCA-associated Vasculitis in Elderly Patients with Renal Immune Complex Deposition

背景抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)多发于老年人,最容易累及肾脏,且多表现为急进性肾小球肾炎,肾脏受累病理特征表现为节段坏死性肾小球肾炎及新月体肾炎,以往认为免疫荧光检查主要提示为寡免疫复合物沉积,但近年来有报道,AAV肾脏免疫荧光上免疫复合物沉积并非罕见。目前,国内很少有关于伴有免疫复合物沉积的AAV研究,对于老年AAV合并有免疫复合物沉积的发病机制、临床、病理特征及对治疗的反应尚不完全清楚。目的探讨合并有肾脏免疫复合物沉积的老年AAV患者的临床与病理特点。方法回顾性分析2009-2018年厦门大学附属中山医院、厦门大学附属第一医院及中国人民解放军联勤保障部队第九〇〇医院肾内科经临床病理确诊的AAV患者56例,根据免疫荧光及电镜免疫复合物的沉积情况,分为免疫复合物沉积组(共14例)和寡免疫复合物沉积组(共42例),两组均使用激素及免疫抑制剂治疗,比较两组的临床表现、多系统受累、实验室检查结果、肾脏病理特点及疗效。结果免疫复合物沉积组和寡免疫复合物组患者性别、年龄、病程、ANCA类型、伯明翰系统性血管炎活动评分系统(BVAS)评分、肾脏受累、肺部受累、皮疹、关节疼痛、中枢神经系统受累发生率、尿素氮(BUN)、血红蛋白(Hb)、白蛋白(ALB)水平、新月体>50%比例、毛细血管袢坏死比例、肾小球硬化比例比较,差异均无统计学意义(P>0.05)。免疫复合物沉积组患者血肌酐(Scr)、24 h尿蛋白水平、新月体比例、间质纤维化比例高于寡免疫复合物组(P<0.05)。免疫复合物沉积组治疗6个月后缓解率为9/14,寡免疫复合物组为76.2%(32/42),两组比较差异无统计学意义(χ^2=0.273,P=0.601)。结论老年AAV患者合并有免疫复合物沉积时提示更为严重的肾损害,肾脏病理上新月体比例更多,间质纤维化程度更高。

Background ANCA-associated vasculitis(AAV)is very common in elderly people and typically affects the kidneys,manifesting as acute glomerulonephritis.The pathological characteristics of renal involvement are segmental necrotizing glomerulonephritis and crescentic nephritis.Previously,immunofluorescence examination was recommended to detect the deposition of oligoimmune complexes.However,in recent years,immune complex deposition in AAV detected by kidney immunofluorescence is not rare.Few studies have examined AAV with immune complex deposition in China.In elderly people,the clinical and pathological characteristics of the pathogenesis of AAV with immune complex deposition,as well as the response to treatment,are not completely clear.Objective To explore the clinical and pathological characteristics of AAV with renal immune complex deposition in elderly patients.Methods We performed a retrospective analysis of 56 AAV patients diagnosed by clinical pathology in Department of Nephrology,Zhongshan Hospital,Xiamen University,the First Affiliated Hospital of Xiamen University,and the 900th Hospital of Joint Logistic Support Force between January 2009 and December 2018.According to the status of immune complex deposition detected by immunofluorescence test and electron microscopy,we assigned 14 with immune complex deposition and 42 with oligoimmunocomplex deposition to immune complex deposition group,and oligoimmunocomplex deposition group,respectively.Both groups were treated with hormones and immunosuppressive agents.We compared the clinical manifestations,multiple system involvement,laboratory results,renal pathological characteristics and treatment response between the groups.Results There were no significant differences between the two groups in sex ratio,mean age,course of disease and BAVAS score,ANCA type distribution,prevalence of kidney,lung,skin,joint muscle and the central nervous system,serum BUN,Hb,ALB level,prevalence of crescents>50%,necrosis of glomerular capillary loop,and glomerulosclerosis(P>0.05).The levels of Scr,24-h urinary protein,the number of crescent bodies,and interstitial fibrosis in the immune complex deposition group were higher than those in the oligoimmune complex group(P<0.05).After 6 months of treatment,the remission rate was 9/14 in the immune complex deposition group and 76.2%(32/42)in the oligoimmune complex group,showing no significant difference(χ^2=0.273,P=0.601).Conclusion Kidney pathology in elderly AAV patients with immune complex deposition often shows more serve renal damage as well as more crescents with a greater degree of interstitial fibrosis.