Baylac-Domengetroy F, Cazenave-Roblot F, Underner M, Boita F, Patte F
Service de Pneumo-Phtisiologie, CHRU de Poitiers.
Rev Pneumol Clin. 1987;43(4):210-5.
This paper is a review of current data on a rare pathology which causes primary pulmonary arterial hypertension: pulmonary veno-occlusive disease. The manifestations of the disease consist of dyspnoea of progressive onset, crepitations in the lower pulmonary lobes and diffuse interstitial syndrome with Kerley's B lines. These signs are associated with severe hypoxia and severe pulmonary arterial hypertension with paradoxically normal wedge pressure. Pathological specimens must be obtained to confirm the diagnosis. They show thrombosis of pulmonary veins less than 2 mm in diameter and sometimes of arterioles, presence of connective tissue with few cells and images of recanalization, muscularization of pulmonary arterioles, lesions of interstitial nodular fibrosis and presence of haemosiderin-rich macrophages. The disease is frequently associated with other pathologies, including heart disease, blood disease and pulmonary capillary haemangiomatosis. In recent years, veno-occlusive disease has been found to occur immediately after chemotherapy for cancer and bone marrow or kidney transplantation. Three physiopathological hypotheses have been put forward to explain the disease: infection, autoimmune reaction and toxic reaction.
本文是对一种导致原发性肺动脉高压的罕见病理——肺静脉闭塞性疾病的当前数据综述。该疾病的表现包括逐渐出现的呼吸困难、下肺叶湿啰音以及伴有克氏B线的弥漫性间质综合征。这些体征与严重缺氧和严重肺动脉高压相关，而肺毛细血管楔压却反常地正常。必须获取病理标本以确诊。病理标本显示直径小于2毫米的肺静脉血栓形成，有时还有小动脉血栓形成，存在细胞稀少的结缔组织和再通图像，肺小动脉肌化，间质结节性纤维化病变以及富含含铁血黄素巨噬细胞。该疾病常与其他病理状况相关，包括心脏病、血液病和肺毛细血管瘤病。近年来，发现静脉闭塞性疾病在癌症化疗后以及骨髓或肾脏移植后立即发生。已提出三种生理病理假说来解释该疾病：感染、自身免疫反应和毒性反应。
