韦格纳肉芽肿病26例临床分析

Clinical analysis of Wegener's granulomatosis:26 cases report

目的分析总结韦格纳肉芽肿病的临床资料,提高对该病的早期诊断及治疗水平。方法收集确诊的26例韦格纳肉芽肿病患者的临床资料,对临床表现、实验室检查、影像学检查、病理学检查等进行回顾性分析。结果 26例中男12例,女14例,发病年龄21～74岁,中位年龄45.3岁。发病至确诊时间为3～48周。首发症状以累及鼻部最多11例(42.3%),临床表现以发热最多17例(65.4%),其次为鼻部症状15例(57.7%),肺部症状14例(53.8%)。2个器官受累3例(11.5%),3个器官受累5例(19.2%),3个以上器官受累18例(69.3%)。24例(92.3%)患者通过病理活检确诊,不同组织病理表现皆为坏死性肉芽肿性炎、炎性细胞浸润性血管炎。胞质型抗中性粒细胞胞浆抗体(cANCA)阳性23例(88.5%),核周型抗中性粒细胞胞浆抗体(pANCA)阳性2例(7.7%)。经糖皮质激素或联合免疫抑制剂治疗效果好。结论韦格纳肉芽肿病的临床表现复杂多样,缺乏特异性,早期易误诊。主要表现为多器官系统受累的症候群,以鼻部、肺脏及肾脏受累最多见,组织活检可确诊,cANCA阳性高度提示本病。早期诊断及治疗有助于改善预后。

Objective To analyse the clinical data of Wegener＇ s granulomatosis,in order to improve the ability of early diagnosis and treatment of the disease.Methods A retrospective study of 26 patients diagnosed as Wegener＇s granulo-matosis was conducted to analyze the clinical manifestations,examination results,pathological and treatment.Results In all patients,12 were males and 14 were females.The ages of patients at disease onset varied from 21 to 74 years.The median age was 45.3 years.Time for diagnosis ranged from 3 to 48 weeks.Patients starting with the symptoms of nasal was most common, for 11 cases（42.3%）;Associated with fever in 17 cases（65.4%）.Two-organ involvement is 3 cases（11.5%）,three-organ involvement is 5 cases（19.2%）,three or more organ involvement in rest patients（69.3%）.All the patients were diagnosed by pathological biopsy,the most common pathological features were necrotizing granulomatous inflammation,and inflammatory cells infiltrated vascularity.Cytoplasmic type anti neutrophil cytoplasmic antibody（cANCA） positive in 23 cases （88.5%）,nuclear weeks type anti neutrophil cytoplasmic antibody（pANCA） positive in 2 patients（7.7%）.The majority of patients accepted glucocorticoid treatment and had good outcomes.Conclusion The clinical manifestations of Wegener＇s granulomatosis is complicated and lack of specificity,easily misdiagnosed in course early.Clinical characteristics of WG is multisystem damage,especially in nasal,lung and kidney,most of patients can diagnose by biopsy,cANCA positive is important of diagnosis before biopsy.Early diagnosis and early treatment is very important to improve prognosis.