血管炎病是血管壁原发的炎症和坏死,导致血管腔狭窄、阻塞或扩张。该病可产生多系统脏器损伤,肺间质是主要受累部位之一。按Chapel Hill会议血管炎分类,大血管炎之一、多发大动脉炎的肺血管炎亚型是一个临床少见病,最终肺动脉高压伴肺纤维化。文献报告Takayasu亚型并肺动脉高压 Vasculitis is a primary inflammation and necrosis of the vessel wall, resulting in stenosis, obstruction or expansion of the vessel lumen. The disease can produce multiple system organ damage, interstitial lung is one of the main affected parts. According to Chapel Hill meeting Vasculitis classification, one of the major vasculitis, polyangiitis polyarteritis subtypes of pulmonary vasculitis is a clinical rare, and ultimately pulmonary hypertension with pulmonary fibrosis. The literature reports Takayasu subtype and pulmonary hypertension