Anaev E Kh, Baranova I A, Belevsky A S
Pirogov Russian National Research Medical University, Moscow, Russia.
Ter Arkh. 2018 Apr 19;90(3):99-106. doi: 10.26442/terarkh201890399-106.
Pulmonary vasculitis is a group of rapidly progressing severe diseases characterized by vascular inflammation, destruction and necrosis of the pulmonary tissue. The pathological process in the lungs varies from diffuse alveolar hemorrhage to inflammation of the parenchyma, pleural effusion, thrombotic and thromboembolic complications. Depending on the size of the affected vessels, vasculites are divided into vasculites of large, medium and small vessels. Most frequently the lung is found in the small vessels vasculitis, including ANCA-associated vasculitis [granulomatosis with polyangiitis (GP), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA)] and the disease is glomerular basement membrane (goodpasture syndrome). Clinical examination of other systems and organs involved in the pathological process, including the skin and kidneys, as well as the detection of autoantibodies can improve approaches to early diagnosis and treatment of vasculitis. Treatment of life-threatening pulmonary bleeding and irreversible damage to organs, especially the kidneys, requires rapid diagnosis of these conditions. Vasculitis is a rare disease with lesions of many organs, and methods of their treatment, including biological, are rapidly developing, which requires the cooperation of doctors of various specialties and specialized centers to achieve better control of the disease.
肺血管炎是一组进展迅速的严重疾病，其特征为肺部组织的血管炎症、破坏和坏死。肺部的病理过程从弥漫性肺泡出血到实质炎症、胸腔积液、血栓形成和血栓栓塞并发症不等。根据受累血管的大小，血管炎可分为大、中、小血管血管炎。最常累及肺部的是小血管血管炎，包括抗中性粒细胞胞浆抗体相关性血管炎[肉芽肿性多血管炎（GPA）、嗜酸性肉芽肿性多血管炎（EGPA）和显微镜下多血管炎（MPA）]以及抗肾小球基底膜病（肺出血肾炎综合征）。对病理过程中涉及的其他系统和器官（包括皮肤和肾脏）进行临床检查以及检测自身抗体，可改善血管炎的早期诊断和治疗方法。对于危及生命的肺出血和器官（尤其是肾脏）的不可逆损害，需要快速诊断这些情况。血管炎是一种累及多个器官的罕见疾病，其治疗方法（包括生物治疗）正在迅速发展，这需要各专科医生和专业中心的合作，以更好地控制该疾病。
