抗中性粒细胞胞质抗体相关性血管炎147例临床特征分析

Analysis of clinical features of 147 cases of ANCA-associated vasculitis

分析ANCA相关性血管炎(AAV)患者的临床特征。方法对2015年2月至2022年2月间在北京协和医院风湿免疫科门诊长期随访的AAV患者进行回顾性研究,总结AAV三个临床亚型的人口学特征、临床表现、复发事件、治疗及预后情况。结果147例患者中肉芽肿性多血管炎(GPA)、显微镜下多血管炎(MPA)和嗜酸性肉芽肿性多血管炎(EGPA)分别有71、45、31例,3种疾病中ANCA阳性率分别为91.5%、95.6%和19.4%(X^(2)=76.68,P<0.01)。上呼吸道和肺脏是GPA和EGPA受累的主要器官,肾脏和下呼吸道MPA受累的主要器官,另外,EGPA患者中心包炎、心肌炎、神经系统受累和血栓发生率高于GPA和MPA(分别为12.9%、9.7%、41.9%和19.4%;X^(2)=8.51、7.13、7.54和0.02,P<0.05)。3组患者中位随访时间分别为43、28、46个月,GPA和EGPA复发比较常见(分别为59.2%和64.5%;X^(2)=11.26,P=0.004),其中肺脏和耳鼻喉是最常见出现复发的器官(GPA为61.9%和40.5%,EGPA为55.0%和50.0%),MPA复发时以肺脏及肾脏表现常见(分别为64.3%和60.0%)。3种疾病患者接受的主要治疗药物有糖皮质激素(95.9%)、环磷酰胺(71.4%)、甲氨蝶呤(54.4%)雷公藤(34.0%)霉酚酸酯(31.3%)、硫唑嘌呤(29.3%)来氟米特(19.0%)利妥昔单抗(19.0%)、他克莫司/环孢素(8.8%)。本研究随访期间总有6例患者死亡(4.1%)。结论AAV临床特征与其他国家相似,复发常见,绝大多数患者需要糖皮质激素联合免疫抑制剂治疗。

Objective To analyze the clinical characteristics of patients with anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).Methods A retrospective study was conducted on AAV patients with long-term follow-up in the rheumatology outpatient clinic of Peking Union Medical College Hospital between February 2015 and February 2022.The demographic characteristics,clinical manifestations,concurrent events,treatment,and prognosis of the three clinical subtypes of AAV were collected and analyzed.Results There were 71,45,and 31 cases of granulomatous polyangitis(GPA),microscopic polyangitis(MPA),and eosinophilic granulomatous polyangitis(EGPA),respectively,among 147 patients.The ANCA positivity rates in the three groups were 91.5%,95.6%,and 19.4%(=76.68,P<0.01),respectively.The upper respiratory tract and lungs were the most frequently affected organs in GPA and EGPA,and the kidneys and lower respiratory tract were the main organs involved in MPA.In addition,cardiac and neurological involvement and thrombosis rates were significantly higher in ECPA patients than in GPA and MPA(12.9%,9.7%,41.9%and 19.4%,respectively;X^(2)=8.51、7.13、7.54.0.02,P<0.05).The median follow-up time for the three groups of patients was 43,28,and 46 months respectively.Relapse was more common in patients with GPA and ECPA(up to 59.2%and 64.5%;X^(2)=11.26,P=0.004),with the lungs and ENT being the most common relapse organs(GPA of 61.9%and 40.5%,ECPA of 55.0%and 50.0%),the lungs and kidneys were the most common manifestations in MPA relapse(64.3%and 60.0%,respectively).The main therapeutic agents were glucocortoid(95.9%),cyclophosphamide(71.4%),methotrexate(54.4%),tripterygium wilfordiz(34.0%),mycophenolate mofetil(31.3%),azathioprine(29.3%),leflunomide(19.0%),rituximab(19.0%),and tacrolimus/cyclosporine(8.8%).There were 6 deaths(4.1%)occurred during the follow-up period of this study.Conclusion The clinical features of AAV are similar to those reported in the literature and relapses are common>he vast majority of patients need to be treated with glucocorticoid combined with immunosuppressive agents.