Rabiller A, Jaïs X, Hamid A, Resten A, Parent F, Haque R, Capron F, Sitbon O, Simonneau G, Humbert M
Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie, Hôpital Antoine Béclère, Université Paris-Sud, Clamart, France.
Eur Respir J. 2006 Jan;27(1):108-13. doi: 10.1183/09031936.06.00054105.
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension that affects predominantly post-capillary pulmonary vessels. A major concern with PVOD is the poor response to available therapies and the risk of pulmonary oedema with continuous intravenous epoprostenol. The present authors hypothesised that alveolar haemorrhage may be a characteristic feature of pulmonary veno-occlusive disease, as compared with other forms of pulmonary arterial hypertension that predominantly involve pre-capillary pulmonary arteries. This paper reports a series of 19 patients with either PVOD (n = 8) or idiopathic pulmonary arterial hypertension (IPAH; n = 11) who underwent bronchoalveolar lavage. Cytological analyses were performed and differential counts were made on Perls-stained preparations. The Golde score was used to assess alveolar haemorrhage. As compared with IPAH, PVOD was characterised by a higher percentage of haemosiderin-laden macrophages (40+/-37 versus 3+/-6%), resulting in elevated Golde scores (81+/-88 versus 4+/-10). It was concluded that occult alveolar haemorrhage is a common feature of pulmonary veno-occlusive disease. Detecting occult alveolar haemorrhage may be of interest in the diagnostic approach of pulmonary veno-occlusive disease.
肺静脉闭塞性疾病(PVOD)是一种罕见的肺动脉高压病因,主要影响肺毛细血管后血管。PVOD的一个主要问题是对现有治疗反应不佳,以及持续静脉输注依前列醇会导致肺水肿。本文作者推测,与主要累及肺毛细血管前动脉的其他形式的肺动脉高压相比,肺泡出血可能是肺静脉闭塞性疾病的一个特征性表现。本文报告了19例接受支气管肺泡灌洗的PVOD患者(n = 8)或特发性肺动脉高压(IPAH;n = 11)患者。进行了细胞学分析,并对Perls染色制剂进行了分类计数。使用Golde评分评估肺泡出血。与IPAH相比,PVOD的特征是含铁血黄素巨噬细胞百分比更高(40±37%对3±6%),导致Golde评分升高(81±88对4±10)。得出的结论是,隐匿性肺泡出血是肺静脉闭塞性疾病的常见特征。在肺静脉闭塞性疾病的诊断方法中,检测隐匿性肺泡出血可能具有重要意义。