韦格纳肉芽肿34例肺损害的临床分析

Wegener's granulomatosis with pulmonary involvement:a report of 34 cases

目的分析韦格纳肉芽肿的临床资料,提高对本病肺损害的认识。方法对34例确诊有肺损害的韦格纳肉芽肿患者的临床资料进行回顾性分析。结果男18例,女16例,从初发到确诊的时间为20天-9年,平均11.1个月。肺部症状:咳嗽、咳痰发生率55.9%,咯血为23.5%,胸痛为14.7%,气短、呼吸困难为14.7%,无症状者为29.4%。肺外可累及多个系统或器官,主要累及上呼吸道和肾脏。胸部影像学表现为:双肺单发或多发结节肿块阴影发生率61.8%,结节性空洞发生率57%,双肺单发或多发磨玻璃阴影发生率14.7%,肺实变阴影发生率14.7%,胸腔积液发生率8.8%。最常见的病理学表现为坏死性肉芽肿性炎和血管炎。抗中性粒细胞胞质自身抗体(ANCA)检查,阳性率91.7%,其中cANCA86.4%,pANCA13.6%。结论肺是韦格纳肉芽肿最易侵犯的器官之一,肺外表现复杂多样,可累及多系统、多器官,胸部影像表现为多发、多形态病灶,容易误诊。组织活检是确诊的金标准,ANCA检查可辅助诊断。

Objective To improve the understanding of Wegener＇s granulomatosis（WG）with pulmonary involvement by analyzing the clinical data of WG.Methods The clinical features of 34 cases of Wegener＇s granulomatosis with pulmonary involvement were retrospectively investigated.Results There were 18 male and 16 female.The ages of WG onset ranged from 13 to 76 years（mean 40.1 years）.The time from onset of the manifestations to WG diagnosis was from 20 days to 9 years（mean 11.1 months）.Pulmonary symptoms：the incidence of cough with expectoration was 55.9%,haemoptysis 23.5%,chest pain 14.7%,breathlessness or dyspnea14.7%,asymptomatic 29.4%.Extra pulmonary multiple system and organs were involved,mainly the upper airways and kidneys.Chest radiogram showed bilateral solitary or multiple pulmonary nodules or masses 61.8%,cavitation in the nodules 57%,solitary or multiple pulmonary ground-glass opacification 14.7%,consolidation 14.7%,pleural effusion 8.8%.The most common pathologic features were necrotizing granulomatous inflammation and vasculitis.Anti-neutrophil cytoplasmic antibody（ANCA）positive rate was 91.7%,cANCA 86.4%,pANCA 13.6%.Conclusion Lung is one of the most commonly involved organ,and extra pulmonary multiple system and organs are involved.Chest radiogram mainly shows bilateral multiple pulmonary lesions and multiple morphologic features,and can be easily misdiagnosed.Biopsy is the gold standard of WG,and ANCA assay can be an important way to diagnose WG.