肺小血管炎的研究进展

肺小血管炎(SVVL)是原发性小血管炎累及肺脏血管的一组疾患.因本病与抗中性粒细胞胞浆抗体(ANCA)密切相关,故称为抗中性粒细胞胞浆抗体相关性小血管炎(AAVS).主要包括:韦格纳肉芽肿病(WG)、显微镜下多血管炎(MPA)、变应性肉芽肿性血管炎(CSS)[1],常可累及多个系统.肺是原发性小血管炎累及频率最高的脏器之一,据报道肺脏受累占75%,仅次于肾脏,且是造成死亡的主要原因.其临床表现复杂多变,缺乏特异性,常常被误诊为其它疾病,延误治疗.而经积极治疗,其生存率明显上升.因此有必要提高对SVVL的认识.

Progress in study of small vessel vasculitis of the lung

Small vessel vasculitis of the lung is a series of diseases of primary small vessel vasculitis with involvement of lung. Because this disease has close relatiohship with antineutrophil cytoplasmic antibody, it is also called antineutrophil cytoplamic antibody associated vasculitis. It invades multisystem including Wegener's granulomatosis, microscopic polyangiit, Churg-Strauss syndrom, and so on. Lung is one of the most often invaded organs in this disease. It is reported the invaded frequency of lung is 75 percent, just lower than kidney. And it is the primary reason that leads to death. Its clinical manifestations are complicated and various, and also lack of specificity, so it is often misdiagnosed as other diseases and the treatment is delayed. If it is diagnosed on time, the survival rate will be improved. It is very necessary to understand more clearly about small vessel vasculitis of the lung.