抗髓过氧化物酶抗体阳性韦格纳肉芽肿病肺损害临床研究.docVIP

抗髓过氧化物酶抗体阳性韦格纳肉芽肿病肺损害临床研究【摘要】 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。? 【关键词】 韦格纳肉芽肿病;肺损害;抗髓过氧化物酶抗体 ?? Pulmonary manifestation of wegeners granulomatosis patients with positive antimyeloperoxidase autoantibodies and clinical analysis JU Yang, Fang Bao-min. Department of Respiratory Medicine, Beijing Hospital of Ministry of Health, Beijing 100730, China? 【Abstract】 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 diagn