以不明原因发热为主要表现的ANCA相关性小血管炎12例临床分析

Clinical analysis of the ANCA associated vasculitides with unknown reasons fever as main manifestations:A report of 12 cases

目的探讨以不明原因发热(FUO)为主要表现的抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎的临床特点。方法收集12例以FUO为主要表现的确诊为ANCA相关性小血管炎的临床资料,分析其临床表现、辅助检查及其对诊断的价值和意义。结果 12例患者中男6例,女6例,年龄22～78(55±22)岁,从起病到明确诊断时发热时间为22～65(38±14)d。12例均表现为不同程度的发热,以低热及中等程度发热为主;8例伴有呼吸系统症状;10例伴有肾脏受累,表现为血尿、蛋白尿及肾功能异常;12例患者均存在不同程度贫血(血红蛋白35～98 g/L),其中合并溶血性贫血3例;白细胞总数正常6例,高于正常6例,血小板均正常。血浆白蛋白除1例正常外,余均低于正常。结论 ANCA相关性小血管炎可以不明原因发热起病,临床表现缺乏特征性,大部分病例在疾病的发展过程中会累及多个系统,肾脏是最易受累器官,其次是呼吸系统。

Objective To explore the clinical features of anti-neutrophil cytoplasmic antibody （ANCA） associated vasculitides with fever of unknown origin（FUO） as main manifestations. Methods Clinical material of 12 cases of ANCA as- sociated vasculitides with FUO as main manifestations were collected. Clinical manifestations, laboratory examination and their diagnostic value were analyzed. Results There were 6 males and 6 females. Mean age was 22 -78（55±22） years old. Fe- ver time from onset to diagnosis confirmation was 22 -65 （38±14） days. 12 cases showed varying degree of fever, mainly low to moderate. 8 cases presented with respiratory symptoms. 10 cases presented with renal involvement, expressed as hematuri- a, proteinuria and renal dysfunction. 12 cases had varying degree of anemia （ hemoglobin from 35 to 98g/L）, in which of 3 cases complicated with hemolytic anemia. WBC counts were normal in 6 cases, and higher in 6 cases. The platelet counts were all normal. The plasma albumin level were lower than normal except one case. Conclusion The ANCA associated vas- culitides may onset with FUO, without special clinical manifestations. Most cases involve more than one organ system during the course of the disease. Kidney is the most vulnerable organ involved, followed by respiratory system.