抗髓过氧化物酶抗体阳性韦格纳肉芽肿病肺损害的临床分析

目的提高对抗髓过氧化物酶（MPO）抗体阳性的韦格纳肉芽肿（WG）病患者的肺损害的认识。方法自2002年3月至2008年1月,对确诊的8例有肺损害的韦格纳肉芽肿病患者的临床表现、实验室检查、影像学特点进行回顾性分析并结合文献进行复习。结果8例患者中,男4例、女4例,平均49.83岁。抗MPO抗体阳性4例,抗蛋白酶3（PR3）抗体阳性4例。抗MPO抗体阳性的WG患者,临床表现为发热4例,咳嗽、咳痰4例、咯血2例、呼吸困难1例、哮喘样发作1例;4例均累及上呼吸道和肾脏;胸部CT显示双肺单发或多发结节影2例、肿块影1例,双肺弥漫斑片影或磨玻璃影4例,肺实变影1例,结节性空洞1例及支气管扩张2例。误诊为肺癌、肺结核、军团菌肺炎、真菌性肺炎者各1例。结论在国人的WG患者中,抗MPO抗体阳性可能并不少见。对临床表现为发热、肺部阴影伴抗MPO抗体阳性的患者应考虑WG的可能。

Pulmonary manifestation of wegener's granulomatosis patients with positive antimyeloperoxidase autoantibodies and clinical analysis

Objective To improve the understanding of clinical features of pulmonary of patients with MPO-ANCA positive wegener granulomatosis(WG).Methods 8 WG patients with pulmonary involvement were from Beijing hospital between 2002-2008.Data were retrospectively analyzed and the relevant literatures were reviewed.Results 8 patients fulfilled the diagnostic criteria of WG.4 were male and 4 were female age ranged from 17 to 68,average 49.83 years.4/8 were MPO-ANCA positive,4/8 were PR3-ANCA positive.In patients with MPO-ANCA,pulmonary symptoms including fever(4/4),cough with expectoration(4/4),hemoptysis(2/4),dyspnea(1/4) and asthmatic outbreak(1/4).Extra pulmonary multiple system and organs were involved,mainly the upper airways and kidney.Chest radiogram showed bilateral solitary or multiple pulmonary nodules(2/4) or masses(1/4),multiple pulmonary ground-glass opacification or massive shadow(4/4),consolidation(1/4),cavitation in the nodules(1/4) and bronchiectases(2/4).4/8 were misdiagnose as lung cancer,tuberculosis,legionella pneumonia and pulmonary fungus disease,respectively.Conclusion Patients with MPO-ANCA positive WG were common in Chinese.When patients with MPO-ANCA positive and present with fever and lung shadows,the diagnosis should be highly suspected.