Assistance Publique Hôpitaux de Paris, Bichat Hospital, Rare Pulmonary Diseases Reference Center, Paris, France.
Institut National de la Recherche Médicale, Unit 1152, DHU FIRE, Paris Diderot University, Paris, France.
Semin Respir Crit Care Med. 2018 Aug;39(4):465-470. doi: 10.1055/s-0038-1669914. Epub 2018 Nov 7.
Pulmonary fibrosis is observed in a substantial number of patients with ANCA-associated vasculitis (AAV), 15% in a recent German series, and may be more frequent in Asian populations. ANCA are usually of anti-MPO specificity and microscopic polyangiitis is the most frequent vasculitis. Pulmonary fibrosis may increase the risk of death in patients with AAV. Treatment for AAV in patients with lung fibrosis should follow the international guidelines for vasculitis. The role of anti-fibrotic drugs (pirfenidone, nintedanib) in this condition is still unknown. Pulmonary fibrosis precedes the diagnosis of AAV or is diagnosed concomitantly in most of the cases. Interestingly, 4% to 35% of patients with pulmonary fibrosis are ANCA-positive, but only 7% to 23% of the patients with pulmonary fibrosis and anti-MPO will develop AAV during follow-up. ANCA positivity may be detected in idiopathic or non idiopathic pulmonary fibrosis. In the absence of vasculitis, the detection of ANCA does not influence the diagnostic work-up of patients with lung fibrosis. If an Idiopathic Pulmonary Fibrosis diagnosis is considered, an anti-fibrotic therapy should be considered, according to local and international guidelines.
肺纤维化在相当数量的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者中被观察到，最近的一项德国系列研究中为 15%，且在亚洲人群中可能更为常见。ANCA 通常具有抗髓过氧化物酶特异性，显微镜下多血管炎是最常见的血管炎。肺纤维化可能增加 AAV 患者的死亡风险。肺纤维化患者的 AAV 治疗应遵循血管炎的国际指南。抗纤维化药物（吡非尼酮、尼达尼布）在这种情况下的作用仍不清楚。在大多数情况下，肺纤维化先于 AAV 诊断或同时诊断。有趣的是，4%至 35%的肺纤维化患者为 ANCA 阳性，但只有 7%至 23%的肺纤维化和抗髓过氧化物酶患者在随访中会发展为 AAV。ANCA 阳性可在特发性或非特发性肺纤维化中被检测到。在没有血管炎的情况下，ANCA 的检测并不影响肺纤维化患者的诊断评估。如果考虑特发性肺纤维化的诊断，应根据当地和国际指南考虑进行抗纤维化治疗。
