101例韦格纳肉芽肿临床分析

Wegener's granuloma: A clinical analysis of 101 cases

目的探讨韦格纳肉芽肿(wegener's granulomatosis,WG)的临床特征及诊治方法。方法回顾性分析101例确诊为WG患者的临床资料。结果男51例,女50例。发病年龄8-75(42.7±16.0)岁。发热、乏力占入院患者的72.3%。全身受累脏器依次为肺脏(67.3%)、耳鼻喉(62.4%)、眼部(47.5%)、肾脏(26.7%)、神经系统(18.8%)、皮肤(14.9%)、肌肉关节(14.9%)、腹部(12.9%)、口腔黏膜(8.9%)、心血管系统(6.9%)。101例中90例进行了病理活检,阳性率为96.7%。97例行抗中性粒细胞胞浆抗体(ANCA)血清学检查,阳性率79.4%。入院前误诊23(22.8%)例。所有患者均接受糖皮质激素及(或)细胞毒免疫抑制剂治疗。病情反复住院2次以上24例(23.8%)。3例死于肺部感染所致的呼吸衰竭,2例因急性肾衰竭而行透析治疗,5例6只眼因治疗不及时失明。其余患者症状均明显缓解。结论 WG侵犯全身多脏器,主要以肺、耳鼻喉及眼部居多,临床表现复杂,早期诊断困难,疾病复发率较高;诊治由多学科协助完成,及时治疗以促进预后。

Objective To study the clinical characteristics,diagnostic and treatment methods of Wegener＇s granuloma（WG）.Methods Clinical data about 101 WG patients were retrospectively analyzed.Results Of the 101 WG patients,51 were males and 50 were females at the age of 8-75 years（42.7±16.0 years）,72.3% of them were admitted to our hospital because of fever and fatigue.The involved organs were lungs（67.3%）,followed by ear,nose,and throat（62.4%）,eyes（47.5%）,kidneys（26.7%）,nervous system（18.8%）,skin（14.9%）,muscle and joints（14.9%）,Abdomin（12.9%）,mucous membrane of mouth（8.9%）,and cardiovascular system（6.9%）.Of the 101 WG patients,90 underwent biopsy with a positive rate of 79.4%,97 received serological test of antineutrophil cytoplasmic antibodies（ANCA） with a positive rate of 79.4%,and 24（23.8%） were misdiagnosed before admission.All the patients were treated with glucocorticoids and/or cytotoxic immunosuppressive agents.Twenty-four patients were admitted to hospital over 2 times due to relapse of the disease.Three patients died of respiration failure due to pulmonary infection,2 patients required dialysis due to acute renal failure,and 5 patients（6 eyes） suffered from visual loss due to delayed treatment.The symptoms of other patients were significantly improved.Conclusion WG involves multiple organs,especially lungs,ear,nose,throat and eyes.Its clinical manifestations are complicated,its early diagnosis is difficult,its recurrence rate is rather high,its diagnosis and treatment require the assist from different departments,and prompt treatment improves its prognosis.