伴有免疫复合物沉积的ANCA相关性小血管炎患者临床及肾脏病理分析

目的：探讨有免疫复合物沉积的ANCA相关性小血管炎（AASV）患者临床及病理特点。方法：回顾性地分析近5年诊断明确并行肾活检的27例患者,根据免疫荧光及电镜分为有免疫复合物沉积组和少免疫复合物沉积组,均使用激素及免疫抑制剂治疗,比较两组的临床、病理特点及疗效、预后。结果：有免疫复合物沉积组14例,少免疫复合物沉积组13例。两组在年龄、性别、ANCA类型、起病至肾活检时间,发热、乏力等非特异性表现及肾外器官受累方面差异均无统计学意义。在血尿、高血压及血肌酐方面亦无明显差异,但有免疫复合物沉积组尿蛋白明显高于后者,差异有统计学意义。对激素及免疫抑制治疗的疗效及预后可能更差,但差异无统计学意义。在病理类型方面,两组均为新月体性肾炎,但有免疫复合物沉积组可合并IgA肾病和膜性肾病。结论：肾脏有免疫复合物沉积提示更多的尿蛋白,可能更差的疗效及预后,病理类型上在新月体性肾炎基础上合并IgA肾病和膜性肾病。

Clinical and Pathological Characteristics of ANCA Associated Systemic Vasculitis with Immune Complex Deposition in Kidney

Objective：To analyze the clinical and pathological characteristics of ANCA associated systemic vasculitis （AASV） with immune complex deposition in kidney.Methods：Patients with AASV,admitted in our hospital in last 5 years,were retrospectively studied.They were divided into two groups,the immune complex deposition group（group Ⅰ）, and the pauci-immune deposition group （group Ⅱ）.They are all treated with prednisone and immunosuppressant.The clinical and pathological characteristics and efficacy and prognosis were compared between the two groups.Results：There were fourteen patients in group Ⅰ and thirteen in group Ⅱ.There was no significant difference in age,gender,type of ANCA,interval between onset of vasculitis renal biopsy,and clinical manifestations such as fever and fatigue between the two groups.There was also no significant difference in extrarenal organ involvement at the time of biopsy, and hematuria,serum creatinine levels,and hypertension.However,patients in group I had significantly more Proteinuria.A trend was also observed for a worse efficacy and prognosis However,the differences did not reach significance.Crescentic glomerulonehpritis can be superimposed with other immune complex-mediated renal diseases such as membranous nephropathy and IgA nephropathy in group Ⅰ.Conclusion：Immunocomplex deposition was associated with a significantly greater degree of proteinuria,and maybe a worse efficacy and prognosis.Crescentic glomerulonehpritis can superimposed with membranous nephropathy or IgA nephropathy in AASV with immune complex deposition.