Pulmonary Veno-occlusive Disease: Entity or Syndrome?
Pulmonary Veno-occlusive Disease: Entity or Syndrome?
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肺静脉闭塞性疾病：实体还是综合征？
DOI：
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发表时间：
1976
期刊：
影响因子：
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通讯作者：
C. Wagenvoort
中科院分区：
文献类型：
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作者：
C. Wagenvoort
肺静脉闭塞性疾病是一种罕见且通常致命的疾病，其中肺静脉和小静脉逐渐闭塞。如果没有肺活检，这种疾病的临床诊断就很困难。如果存在肺动脉高压，并伴有肺水肿和充血的 X 线征象，但没有左心房压力升高的征象，则必须考虑诊断。肺部的形态学图像具有特征性。小静脉，有时还有大静脉，被纤维组织变窄或闭塞，几乎肯定是由于有组织的血栓。充血、间质纤维化和肺炎的结节性区域经常出现。在许多病例中已提出病毒病因。然而，如果我们假设肺静脉血栓形成是最初的事件，则必须考虑到这种可能性可能是由某些患者的病毒引起的，而另一些患者则是由毒性因素或凝血障碍引起的。那么，肺静脉闭塞性疾病很可能是一种综合征，而不是一个病因实体。
Pulmonary veno-occlusive disease is a rare and usually fatal condition in which there is gradual obliteration of the pulmonary veins and venules. Without a lung biopsy the clinical diagnosis of this disease is difficult. If there is pulmonary hypertension with roentgenographic signs of pulmonary edema and of congestion in the absence of signs of increased left atrial pressure, the diagnosis must be considered. The morphologic picture of the lungs is characteristic. The small veins, and sometimes also the major veins, are narrowed or occluded by fibrous tissue, almost certainly on the basis of organized thrombi. Nodular areas of congestion, interstitial fibrosis, and pneumonitis are regularly present A viral etiology has been suggested in a number of cases. If we may assume, however, that thrombosis of pulmonary veins is the initial event the possibility has to be considered that this may be elicited by a virus in some patients and by toxic factors or by clotting disorders in others. Pulmonary veno-occlusive disease might then well be a syndrome rather than an etiologic entity.