原发性小血管炎患者多系统临床表现和肾脏病理分析

目的分析原发性小血管炎患者多系统的临床表现和肾脏病理特点。方法选取我院1996年1月至2007年8月间明确诊断的原发性小血管炎患者共78例,患者均有肾脏损害,并行肾活检,总结分析患者的临床病理资料。结果78例中15例胞浆型抗中性粒细胞胞浆抗体(cANCA)阳性,均识别蛋白酶3(PR3);62例环核型抗中性粒细胞胞浆抗体(PANCA)阳性,均识别髓过氧化物酶(MPO)。临床表现呈多器官受累:不规则发热;浮肿、血尿、蛋白尿、肾功能减退;咳嗽、咯血、胸痛、肺部浸润;鼻塞伴有脓性分泌物;关节肿痛等。肾活检可见小血管壁节段性纤维素样坏死,新月体多见。免疫荧光无免疫球蛋白或微量免疫球蛋白及补体C3沉积。治疗采用糖皮质激素联用细胞毒药物或吗替麦考酚酯,部分患者同时给予透析治疗,治疗有效率达85.9%。结论原发性小血管炎并不少见,且以显微镜下型多血管炎居多;患者以中老年为主;ANCA检测有助于早期诊断及复发判断;及时治疗可逆转。

Analysis of multi-system involvement and renal pathology of primary small-vessel vasculitides

Obiective To analyze the clinical and pathological characteristics of primary small-vessel vasculitides.Methods Totally 78 patients with primary small-vessel vasculitides diagnosed in the recent 11 years in our center were studied,and all of their clinical and renal pathological data were analyzed.Results Of the 78 patients,15 were cytoplasmic ANCA (cANCA) positive,and all of them were recognized proteinase 3 (PR3);62 were perinuclear ANCA (cANCA) positive,and all of them were recognized myeloperoxidase (MPO).The clinical manifestations were non-specific symptoms of multisystem involvements which included irregular fever;edema and hematuria;proteinuria and renal hypofunction;cough,hemoptysis,thoracalgia and lung infiltration;nasal obstruction and purulent secretion;joint swelling and painfulness.The renal specimen biopsy demonstrated focal necrotizing and crescentic glomerulonephritis.No immunoglobulin or skeptophylaxis immunoglobulin or complement C3 was detected in immunofluorescent microscopic findings.The curative effect was 85.9% when glucocorticoid was used in combination with cyclophophamide or MMF in the treatment while some of the patients received dialysis.Conclusion Primary small-vessel vasculitides (ANCA related small-vessel vasculitides) were commonly found in China,the microscopic type multi-vessel vasculitides were even more common in clinic.The majority of the patients were elderly people.An ANCA test is helpful in early diagnosis and recurrence determination,and timely treatment can reverse it.