老年人抗中性粒细胞胞浆抗体相关性小血管炎的临床表现

Clinical manifestations in elderly patients with ANCA-related vasculitis

目的 为了解老年人抗中性粒细胞胞浆抗体 (ANCA)相关性小血管炎所引起的多器官损害的临床表现。方法 分析总结近 5年来检出的 1 4 5例 60岁以上老年ANCA相关性小血管炎患者的临床病理资料。结果 1 4 5例患者 ,男 74例 ,女 71例 ,平均年龄 68.2岁。胞浆型ANCA(c ANCA)阳性 1 7例 ,均识别蛋白酶 3(PR3) ;环核型ANCA (p ANCA)阳性 1 2 8例 ,其中 1 2 0 1 2 8例单独识别髓过氧化物酶 (MPO) ,8 1 2 8例同时识别PR3和MPO。不足 1 2的患者在 3个月内确诊。确诊病例均呈多器官损害 ,其中肾脏受累占 93 .8% ,且 75 %出现肾功能不全 ;肺脏受累占 74.5 % ,半数以上表现为肺出血和 (或 )肺部阴影 ,此外还有不同程度的消化道、神经系统、肌肉关节、眼、耳器官或组织损害及发热、乏力、消瘦等非特异症状。实验室检查 ,90 %以上的患者有中重度贫血、血沉增快和C反应蛋白升高。结论 老年人ANCA阳性小血管炎较为多见 ,临床表现为多器官损害 。

Objective To analyze the multiple organ damage in elderly patients with antineutrophil cytoplasmic antibody(ANCA) related vasculitis. Methods Clinical and pathological data of 145 ANCA positive vasculitis patients over 60 years old diagnosed in recent five years were analyzed. Results Of the 145 patients, 74 were male and 71 were female with an average age of 68.2 years old.Seventeen of the 145 patients were cytoplasmic ANCA (c ANCA) positive and all recognized the proteinase 3 (PR3). The other 128 were perinuclear ANCA (p ANCA) positive and 120/128 recognized myeloperoxidase(MPO), 8/128 recognized both PR3 and MPO.Less than 50% of the patients were correctly diagnosed within 3 months. Clinically,75.8% of the patients had fever and fatigue; 45.1% had body weight loss; 93.8% had kidney involvement and 75% of them had renal failure; 74.5% had lung involvement, over half of them had hemoptysis or lung infiltrates,some patients also had pulmonary fibrosis ,nodules or cavities. Other clinical manifestations included arthralgia (46.9%), myalgia (38.6%), gastrointestinal symptoms (38.6%),eye involvement(27.6%), and ENT involvement (30.3%). In laboratory examinations, 94.4% of the patients had anemia; 62.4% had increased WBC count; 93.6% had increased ESR, and 55.1% had increased C reactive protein(CRP). Conclusions ANCA related vasculitis was common in the elderly.Kidney was the most vulnerable organ to be affected and lung is the most important extrarenal organ to be involved. For elderly patients with multi organ damage, an ANCA test should be performed in order to make an early diagnosis and start therapy in time.