Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine-Béclère, Assistance Publique - Hôpitaux de Paris, Université Paris-Sud 11, INSERM U999, Centre National de Référence de l'Hypertension Pulmonaire Sévère, 92140 Clamart, France.
Respir Med. 2010 Jul;104 Suppl 1:S23-32. doi: 10.1016/j.rmed.2010.03.014. Epub 2010 Apr 24.
Pulmonary veno-occlusive disease (PVOD) is an uncommon form of pulmonary arterial hypertension characterised by a progressive obstruction of small pulmonary veins that leads to elevation in pulmonary vascular resistance and right ventricular failure. Despite improved understanding and more efficacious treatment options for PAH overall, the prognosis of PVOD remains dismal. Without therapeutic intervention few patients would be expected to survive more than two years. PVOD may occur in both idiopathic and heritable forms, or develop in association with connective tissue disease, chronic respiratory disease, malignancy or bone marrow transplantation, among other causes. A widespread fibrous intimal proliferation that predominantly involves the pulmonary venules and small veins is the key histopathological hallmark. Surgical lung biopsy is considered the definitive diagnostic test but is associated with significant risk and is not recommended. Distinguishing PVOD from PAH on clinical grounds alone is generally not possible, although PVOD is characterised by a higher male/female ratio and higher tobacco exposure. Instead, non-invasive tests may be helpful and the diagnosis is usually based on an integrated assessment that incorporates high resolution computed tomography (septal lines, ground-glass opacities and lymph node enlargement), pulmonary function testing (lower DLCO), arterial blood gas analysis (lower PaO(2) at rest) and bronchoalveolar lavage (occult alveolar haemorrhage). Treatment of PVOD remains challenging as exposure to pulmonary vasodilators and PAH-specific agents may precipitate acute pulmonary oedema. Nonetheless, a number of successful outcomes describing cautious use of prostanoids, endothelin antagonists and phosphodiesterase type-5 inhibitors have been described. Unfortunately, the long term effects of these agents are variable and lung transplantation remains the treatment of choice.
肺静脉闭塞病（PVOD）是一种罕见的肺动脉高压形式，其特征是小肺静脉渐进性阻塞，导致肺血管阻力升高和右心衰竭。尽管对肺动脉高压的总体认识有所提高，并且治疗选择更加有效，但 PVOD 的预后仍然不佳。如果没有治疗干预，很少有患者能够存活两年以上。PVOD 可能发生在特发性和遗传性形式中，或与结缔组织病、慢性呼吸道疾病、恶性肿瘤或骨髓移植等其他原因相关。广泛的纤维内膜增生主要累及肺小静脉和小静脉，是关键的组织病理学特征。外科肺活检被认为是明确的诊断测试，但风险很大，不建议进行。仅凭临床症状区分 PVOD 和 PAH 通常是不可能的，尽管 PVOD 的男女比例更高，吸烟暴露更多。相反，非侵入性测试可能会有所帮助，诊断通常基于综合评估，包括高分辨率计算机断层扫描（间隔线、磨玻璃影和淋巴结肿大）、肺功能测试（更低的 DLCO）、动脉血气分析（休息时更低的 PaO2）和支气管肺泡灌洗（隐匿性肺泡出血）。PVOD 的治疗仍然具有挑战性，因为暴露于肺动脉扩张剂和肺动脉高压特异性药物可能会引发急性肺水肿。尽管如此，已经描述了许多成功的病例，描述了谨慎使用前列环素、内皮素拮抗剂和磷酸二酯酶 5 抑制剂。不幸的是，这些药物的长期效果是可变的，肺移植仍然是首选治疗方法。
