Hesse Christian, Kovacs Gabor, Scheidl Stefan, Olschewski Horst
Division of Pulmonology, Medical University of Graz, Graz, Austria.
Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria.
SAGE Open Med Case Rep. 2024 Nov 21;12:2050313X241292529. doi: 10.1177/2050313X241292529. eCollection 2024.
Pulmonary veno-occlusive disease has a significantly worse prognosis than idiopathic pulmonary arterial hypertension. According to a case series from France, the median survival time from diagnosis to death or lung transplantation was only 1 year, and in a more recent analysis, pulmonary arterial hypertension therapy had no significant effect on survival. There are case reports and case series describing both beneficial and adverse effects of pulmonary arterial hypertension-related medications. The most life-threatening complication of such a therapy is pulmonary oedema. In the long term, lung transplantation remains the best treatment option for suitable patients. However, elderly patients with concomitant or precipitating malignant disease are not considered transplant candidates. We describe a 59-year-old pulmonary veno-occlusive disease patient with multiple myeloma in World Health Organisation functional class IV who was successfully treated with sildenafil for almost 5 years.
肺静脉闭塞性疾病的预后明显比特发性肺动脉高压更差。根据法国的一个病例系列，从诊断到死亡或肺移植的中位生存时间仅为1年，并且在最近的一项分析中，肺动脉高压治疗对生存没有显著影响。有病例报告和病例系列描述了与肺动脉高压相关药物的有益和不良影响。这种治疗最危及生命的并发症是肺水肿。从长远来看，肺移植仍然是适合患者的最佳治疗选择。然而，伴有或引发恶性疾病的老年患者不被视为移植候选者。我们描述了一名59岁的世界卫生组织功能分级为IV级的肺静脉闭塞性疾病患者，患有多发性骨髓瘤，他成功地接受了西地那非治疗近5年。
