抗中性粒细胞胞浆抗体相关小血管炎26例临床分析

目的研究抗中性粒细胞胞浆抗体(ANCA)相关小血管炎(AASV)的临床特征、诊断与治疗,以进一步提高对该病的认识。方法回顾分析我院2003～2007年确诊的26例AASV患者的临床资料,总结并分析AASV的临床及实验室特征、诊断方法和治疗。结果26例病例中男17例,女9例,平均年龄62(18～80)岁,其中60岁以上者18例(占69.2%)。cANCA阳性7例,均识别PR3;pANCA阳性18例,均识别MPO;1例ANCA阴性。10例行病理检查,5例有典型的血管炎表现,如小血管局灶节段性纤维素样坏死、坏死性肉芽肿等。以肾、肺受累最为常见,受累率分别为88.5%和73.1%。80.8%有发热、乏力、纳差、消瘦等全身症状,并且是最常见的首发表现。多数病人有贫血、低蛋白血症、白细胞(WBC)及血小板(PLT)计数升高、血沉(ESR)快、C反应蛋白(CRP)及免疫球蛋白增高、类风湿因子(RF)阳性等实验室表现。92.3%病例入院前误诊,误诊的疾病多种多样。多数患者接受激素和环磷酰胺治疗后病情明显改善。结论AASV好发于老年男性,临床表现呈多器官受累,复杂多样,首发表现和实验室检查缺乏特异性,常被误诊。ANCA检测及病理活检有利于本病早期诊断。联合免疫治疗可取得较好的疗效。

The clinical analysis of 26 patients with anti-neutrophil cytoplasmic autoantibodies-related vasculitis

Objective To study the clinical feature,diagnosis and treatment of anti-neutrophil cytoplasmic autoantibodies（ANCA）associated systemic vasculitis（AASV）so as to improve the understanding of this disorder.Methods 26 patients with AASV diagnosed in our center from 2003 to 2007 were retrospectively studied and their clinical data were analyzed.Results Of the 26 patients,17 were male and 9 were female with an average age at 62（18～80）years old.7 of the 26 were cytoplasmic ANCA（cANCA）positive,all of them recognized proteinase 3（PR3）;18 of the 26 were perinuclear ANCA（pANCA）positive,all of them recognized myeloperoxidase（MPO）.Only 1 were ANCA negative.Ten patients underwent tissue biopsy.Five patients biopsies showed typical manifestation of small vessel vasculitis,such as focal segmental necrotizing vasculitis and necrotizing granulomatous inflammation.The most common involved systems are kidney（88.5%）and lung（73.1%）.Other clinical manifestations included nervous system disorder（38.5%）,upper respiratory tract involvement（46.2%）,ophthalmic involvement（30.8%）,joints and muscle involvement（46.2%）,skin involvement（42.3%）,cardiac involvement（26.9%）and gastrointestinal symptoms（23.1%）.80.8% patients had general symptoms such as fever,fatigue,anorexia,weight loss,which were the most common initial manifestations.In laboratory examinations,most patients had anemia,hypoproteinemia,an elevated WBC and PLT count,ESR,CRP,Immunoglobulin,RF ect.The rate of pre-admission misdiagnosis were 92.3%.They had been diagnosed as multiple diseases by error.Most patients improved significantly after being treated with glucorticosteroid and cyclophosphamide.Conclusions Patients with AASV were mainly old male with multi-system damage,the first manifestation and laboratory examinations have poor specificity.So it was frequently misdiagnosed.An ANCA test and biopsy should be helpful for early diagnosis of AASV.Corticosteroid and immunosuppressive treatment may achieve good outcome.