Mouly S, Brillet G, Stern M, Lesavre P, Guillevin L
Department of Nephrology, Necker Hospital, Paris, France.
Scand J Rheumatol. 2000;29(5):333-5.
The association of lung emphysema with severe systemic antineutrophil cytoplasm antibodies (ANCA)-positive vasculitis, such as Wegener's granulomatosis is unusual since only four cases have been described previously. We report the first case of a 30 year-old smoker man presenting with biopsy-proven Wegener's granulomatosis, who developed a bullous emphysema during severe active lung vasculitis, in association with positive ANCA disclosing an anti-myeloperoxydase pattern. Alpha 1-antitrypsin deficiency, a known risk factor of lung emphysema recently found to be associated with anti-proteinase 3-positive vasculitis, was not present in this patient. Cigarette smoking, in association with severe lung vasculitis, might have contributed to the development of this emphysematous lesion.
肺气肿与严重的系统性抗中性粒细胞胞浆抗体（ANCA）阳性血管炎（如韦格纳肉芽肿）相关的情况并不常见，因为此前仅报道过4例。我们报告首例病例，一名30岁男性吸烟者，经活检证实患有韦格纳肉芽肿，在严重的活动性肺部血管炎期间出现大疱性肺气肿，同时ANCA阳性，显示抗髓过氧化物酶模式。该患者不存在α1-抗胰蛋白酶缺乏，这是肺气肿的一个已知危险因素，最近发现其与抗蛋白酶3阳性血管炎有关。吸烟与严重的肺部血管炎可能共同促成了这种肺气肿病变的发生。
