426例抗中性粒细胞胞浆抗体相关性小血管炎患者多系统临床表现和肾脏病理分析

An analysis of multi-system involvement and renal pathology of anti-neutrophil cytoplasmic autoantibodies-related vasculitis

目的分析426例抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎患者多系统的临床和病理表现。方法回顾性分析我院1997年-2004年6月检测并明确诊断的426例ANCA相关性小血管炎患者的临床病理资料。结果426例患者中,70例胞浆型ANCA(cANCA)阳性,均识别蛋白酶3(PR3);354例环核型ANCA(pANCA)阳性,均识别髓过氧化物酶(MPO)。201例(47.2%,201/426)患者是在发病后3个月内确诊。临床表现呈多器官受累,其中cANCA阳性者皮疹、关节痛、眼、鼻受累的发生率显著高于pANCA阳性者,而pANCA阳性者肾脏受累和乏力的发生率显著高于cANCA阳性者。多数患者有贫血,血沉增快,C反应蛋白增高。采用糖皮质激素联合环磷酰胺进行强化免疫抑制治疗,诱导缓解期的缓解率为88.5%。结论ANCA相关性小血管炎在我国并非少见,临床表现呈多器官受累,ANCA检测有助于早期诊断。

Objective To analyze the clinical and pathological characteristics of anti-neutrophil cytoplasmic autoantibodies （ANCA） associated systemic vasculitis （AASV）. Methods 426 patients with AASV diagnosed in recent 8 years in our center were retrospectively studied and their clinical and pathological data were analyzed. Results Of the 426 patients, 70 were cytoplasmic ANCA （ cANCA ） positive, all of them recognized proteinase 3 （PR3） ; 354 were perinuclear ANCA （pANCA） positive, all of them recognized myeloperoxidase （MPO）. Only 47.2% （201/426） of the patients were diagnosed within three months. Clinically, 87. 1% （371/426） had renal involvement and 68.0% （83/122） had crescentic glomerulonephritis. 61.0% （260/426） had lung involvement. Other clinical manifestations included gastrointestinal symptoms （43. 0% ）, nervous system disorder （ 15.7% ）, ophthalmic （ 19. 5% ）, otologic （23.2%） and nasal involvement （8.2%）. The prevalences of fatigue and renal involvement were significantly higher in patients with pANCA than those in patients with cANCA （63.8% vs 47. 1%, P 〈 0. 01 ; 88.7% vs 78. 6% , P 〈 0. 05, respectively）. The prevalences of ophthalmic, nasal invlovement, skin rash and axthragia were significantly higher in patients with cANCA than those in patients with pANCA （31.4% vs 16.9%, P〈0.01; 21.4% vs 5.6, P〈0.01; 28.6% vs 16.4%, P〈0.05; 44.3% vs 28.0%, P 〈 0. 01, respectively）. The patients were treated with corticosteroid and cyclophosphamide. The remission rate in induction phase was 88.5%. Conclusions AASV is a common autoimmune disease in Chinese. For patients with multi-system damage, an ANCA test should be performed in order to make an early diagnosis and start therapy in time.