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目的总结老年ANCA相关性小血管炎的临床特点并分析其死亡相关因素。方法回顾性分析2013年1月至2016年5月于我院住院明确诊断为ANCA相关性小血管炎的60岁以上老年患者共58例,收集患者的一般临床资料及预后情况。结果患者男38例(65.5%),女20例(34.5%);平均年龄(72.47±8.20)岁,80岁以上患者占27.6%。首诊科室分散在7个科室。临床表现为多系统受累,首发症状中最常见为咳嗽咳痰(31.0%),其次为发热(19.0%)。抗MPO-ANCA阳性者42例(72.4%),抗PR3-ANCA阳性者11例(19.0%),同时识别者5例(8.6%)。单纯抗PR3-ANCA阳性患者鼻部受累及神经系统受累的发生率显著高于单纯抗MPO-ANCA阳性患者(鼻部受累:45.5%vs.7.1%,P=0.007;神经系统受累:36.4%vs.4.8%,P=0.016)。在58例AAV患者中,有完整随访资料的有33例,其中17例死亡,均与肺部感染、呼吸衰竭相关,3例肺曲霉菌病患者均死亡,9例接受甲泼尼龙冲击治疗的患者中有随访资料的5例全部死亡。死亡患者中肺间质病变的发生率(88.2%)显著高于存活患者(31.3%),两者差异有统计学意义(P=0.001)。结论老年ANCA相关性小血管炎首发症状多样、多系统受累、就诊科室分散,接受甲泼尼龙冲击治疗、合并肺间质纤维化提示死亡风险更高。
Objective To summarize the clinical features of elderly ANCA-associated vasculitis and analyze its related factors of death. Methods A total of 58 elderly patients over 60 years of age with definite diagnosis of ANCA-associated vasculitis in our hospital from January 2013 to May 2016 were retrospectively analyzed. The general clinical data and prognosis were collected. Results There were 38 males (65.5%) and 20 females (34.5%) with an average age of (72.47 ± 8.20) years and 27.6% of patients over the age of 80 years. Discipline department first scattered in seven departments. Clinical manifestations of multiple system involvement, the most common symptoms of cough and sputum (31.0%), followed by fever (19.0%). 42 (72.4%) were anti-MPO-ANCA positive, 11 (19.0%) were anti-PR3-ANCA positive and 5 (8.6%) were identified at the same time. The incidence of nasal involvement and nervous system involvement was significantly higher in patients with anti-PR3-ANCA alone than in those with anti-MPO-ANCA alone (nasal involvement: 45.5% vs.7.1%, P = 0.007; nervous system involvement: 36.4% vs .4.8%, P = 0.016). Of 58 AAV patients, 33 had complete follow-up data, of which 17 were fatal, both associated with pulmonary infection and respiratory failure, 3 with pulmonary aspergillosis and 9 with methylprednisolone All 5 patients with follow-up data died. The incidence of interstitial lung disease (88.2%) was significantly higher in survivors than in survivors (31.3%), the difference was statistically significant (P = 0.001). Conclusions The elderly ANCA-associated vasculitis has various initial symptoms and multiple system involvement. The department of treatment is scattered and treated with methylprednisolone. The risk of death is higher with pulmonary interstitial fibrosis.