30例以发热症状首诊的ANCA相关性血管炎临床诊治分析

Analysis of clinical diagnosis and treatment in 30 cases of ANCA-associated vasculitis with fever as primary symptom

目的总结以发热为首发症状的抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的临床特征,以提高早期诊断能力,减少漏诊及误诊。方法回顾性分析2016年1月至2021年12月该院收治的以发热症状首诊的30例AAV患者临床资料,总结患者的主要症状、实验室检验结果、肺部CT特征、首诊科室、治疗及转归。结果30例AAV患者中,髓过氧化物酶(MPO)-ANCA阳性22例(73.3%),丝氨酸蛋白酶3(PR3)-ANCA阳性8例(26.7%),MPO-ANCA阳性患者平均年龄明显高于PR3-ANCA阳性患者(P=0.006)。首诊科室以感染科(33.3%)为主,其次为风湿科(30.0%)和呼吸科(16.7%)。共26例患者发生误诊,误诊率为86.7%。90.0%的患者出现肺部影像学异常,66.7%的患者合并尿潜血阳性,40.0%的患者合并血清肌酐升高。所有患者均给予抑制免疫治疗后,除2例患者病情加重,28例患者均取得较好的治疗效果,病情稳定出院。结论发热症状首诊的AAV临床表现缺乏特异性,易误诊,对于发热合并多脏器功能受损且抗感染效果不佳者,需及时行血清ANCA或病理组织学检查,以早诊断、早治疗,减少误诊、漏诊发生,改善患者预后。

Objective To summarize the clinical characteristics of anti-neutrophil cytoplasmic autoantibodies(ANCA)associated vasculitis(AAV)with fever as the initial symptom in order to increase early diagnostic ability and reduce the missed diagnosis and misdiagnosis.Methods The clinical data of 30 AAV patients with fever as the initial symptom in this hospital from January 2016 to December 2021 were retrospectively analyzed.The main symptoms,laboratory tests results,lung CT imaging characteristics,first visiting department,treatment and outcomes were summarized.Results Among 30 cases of AAV,22 cases(73.3%)were positive for myeloperoxidase(MPO)-ANCA,and 8 cases(26.7%)were positive for PR3-ANCA.The average age in the patients with MPO-ANCA positive was greater than that in the patients with PR3-ANCA positive(P=0.006).The first visited departments were dominated by the infection department(33.3%),followed by the rheumatism department(30.0%)and respiratory department(16.7%).A total of 26 cases were misdiagnosed,with a misdiagnosis rate of 86.7%.Among all the patients,90.0%of them appeared the pulmonary imaging abnormality,66.7%of them complicated with urine occult blood positive,and 40.0%of them complicated with serum creatinine increase.All patients were given immunosuppressive agents.Except for 2 patients with aggravated disease,the other 28 patients achieved good effect,and were discharged from hospital in a stable condition.Conclusion The clinical manifestations of AAV with fever as the initial symptom are lack of specificity and easy to be misdiagnosed.For the patients with fever complicated with multiple organ dysfunction and poor anti-infection effect,ANCA or histopathological examination should be conducted in time to conduct early diagnosis and early treatment,so as to reduce the occurrence of misdiagnosis and missed diagnosis and improve the prognosis of the disease.