Pulmonary veno-occlusive disease
Pulmonary veno-occlusive disease
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DOI：
10.1183/09031936.00090608
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发表时间：
2009-01-01
影响因子：
24.3
通讯作者：
Humbert, M.
中科院分区：
文献类型：
--
作者：
Montani, D.;Price, L. C.;Humbert, M.
肺静脉闭塞性疾病（PVOD）目前被归类为肺动脉高压（PAH）的一个亚组，在最初被认为是特发性PAH的病例中占5-10%。PVOD被描述为特发性或并发其他病症，包括结缔组织疾病、艾滋病毒感染、骨髓移植、结节病和肺朗格汉斯细胞肉芽肿病。PVOD与PAH具有广泛相似的临床表现、遗传背景和血流动力学特征。与多环芳烃相比，PVOD的特点是男性/女性比例更高，烟草暴露量更高，静息时动脉血氧压更低，肺对一氧化碳的扩散能力更低，6分钟步行测试时的血氧饱和度最低点更低。胸部高分辨率计算机断层扫描（HRCT）可提示PVOD存在小叶中心磨玻璃影，间隔线和淋巴结肿大。同样，隐匿性肺泡出血与PVOD有关。采用胸部HRCT、动脉血气、肺功能检查和支气管肺泡灌洗等无创诊断方法有助于发现PVOD患者，并避免高风险的手术肺活检以进行组织学证实。PVOD的特点是预后差，并有可能发展为严重的肺水肿与特定的多环芳烃治疗。肺移植是治疗的首选。然而，谨慎使用特定的多环芳烃治疗对某些患者是有帮助的。
Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.