Hospices Civils de Lyon, Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Lyon, France.
Claude Bernard Lyon 1 University, University of Lyon, INRA, UMR754, Lyon, France.
Respiration. 2018;96(1):12-28. doi: 10.1159/000486899. Epub 2018 Jul 4.
The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.
呼吸系统可能参与所有类型的系统性血管炎，其意义和频率各不相同。ANCA 相关性血管炎，包括肉芽肿性多血管炎、嗜酸性肉芽肿性多血管炎和显微镜下多血管炎，比其他血管炎类型更常影响呼吸道。肉芽肿性多血管炎总是与上或下呼吸道受累有关。肺部和耳鼻喉受累是该疾病的标志性特征，分别存在于 90%和 80%的病例中，常伴有皮肤或胃肠道受累。在大约 10%的病例中，肺部是唯一受影响的器官。嗜酸性肉芽肿性多血管炎总是与嗜酸性粒细胞增多和哮喘有关，哮喘通常先于系统性表现数年出现；然而，哮喘和血管炎的发病可能是同时的。实质浸润可能是迁移性的，并在皮质类固醇治疗后迅速消退。弥漫性肺泡出血和肾衰竭是显微镜下多血管炎的典型特征。前者是抗肾小球基底膜病的主要表现，通常是肺-肾综合征的一部分。Takayasu 动脉炎由于肺血管炎而具有独特的临床表现，可表现为大咯血、胸痛，且很少出现肺动脉高压的症状。白塞病是肺动脉瘤的最常见原因，也可导致肺动脉原位血栓形成。皮质类固醇和免疫抑制剂是主要的治疗方法。总之，系统性血管炎是呼吸系统受累的常见原因，其表现多样，严重程度和预后各不相同。
