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

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

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。

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（d/ 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.