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[目的]分析抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的临床表现及治疗。[方法]对我院2005年4月～2010年4月收治的47例AAV患者的临床资料进行回顾性分析。[结果]本组47例患者中,38例(80.9%,38/47)环核型ANCA(pANCA)阳性,均识别髓过氧化物酶(MPO),8例(17.0%,8/47)胞浆型ANCA(cANCA)阳性,均识别蛋白酶3(PR3),1例cANCA及pANCA均阳性,同时识别MPO及PR3。6个月内确诊者27例(57.4%,27/47),6个月以上确诊者20例(42.6%,20/47)。临床表现呈多器官受累,其中肾脏受累43例(91.5%,43/47),血肌酐升高36例(76.6%,35/47),肺脏受累30例(63.8%,30/47),咯血16例(34.0%,16/47)。此外还有不同程度的消化系统、关节、肌肉、耳、眼、皮肤、神经系统、鼻等器官损害及非特异性表现。80%以上患者有血沉增快、C反应蛋白升高及贫血。主要采用糖皮质激素联合环磷酰胺治疗,诱导缓解期总缓解率75.9%。[结论]抗中性粒细胞胞浆抗体(ANCA)相关性血管炎临床表现为多器官受累,ANCA检测有助于早期诊断。
[Objective] To analyze the clinical manifestation and treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). [Methods] The clinical data of 47 patients with AAV treated in our hospital from April 2005 to April 2010 were retrospectively analyzed. [Results] Among the 47 patients, 38 (80.9%, 38/47) were positive for cytoplasmic ANCA (pANCA), all of which were identified as myeloperoxidase (MPO). Eight cases (17.0%, 8/47) Positive cytoplasmic ANCA (cANCA), both positive for protease 3 (PR3), positive for cANCA and pANCA in one case, 27 (57.4%, 27/47) and 6 20 cases were diagnosed over the month (42.6%, 20/47). There were 43 patients (91.5%, 43/47) with renal involvement, 36 (76.6%, 35/47) patients with elevated serum creatinine, 30 (63.8%, 30/47) with lung involvement, 16 cases (34.0%, 16/47). In addition there are varying degrees of digestive system, joints, muscles, ears, eyes, skin, nervous system, nose and other organ damage and non-specific performance. More than 80% of patients have ESR, C-reactive protein and anemia. The main use of glucocorticoid combined with cyclophosphamide treatment, induction of remission, the total remission rate of 75.9%. [Conclusion] The clinical manifestations of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis are multiple organ involvement. ANCA may be helpful for early diagnosis.