A definite diagnosis of pvod thus requires a lung biopsy or pathologic examination of. Pulmonary venoocclusive disease circulation aha journals. Similarities between this and other cases in the literature suggest a possible association between the crest variant of scleroderma and pulmonary veno occlusive disease. Pulmonary veno occlusive disease is a rare cause of pulmonary hypertension. Incidence of anicteric venoocclusive diseasesinusoidal. Recently, biallelic mutations in the eukaryotic translation initiation factor 2. Pulmonary venoocclusive disease in an adult following. Pubmed is a searchable database of medical literature and lists journal articles that discuss pulmonary venoocclusive disease. Krichman am, farber hw, frost ae, barst rj, benza rl, liou tg, turner m, giles s, feldkircher k, miller dp, mcgoon md. Pulmonary veno occlusive disease pvod was diagnosed in an adult following chemotherapy and bone marrow transplantation bmt for acute lymphoblastic leukemia.
One of the prominent changes of the recent guidelines was to move pulmonary venoocclusive disease pvod and pulmonary capillary haemangiomatosis from separate categories into a single subcategory of pah group 1. Longterm survivor with pulmonary venoocclusive disease. Pulmonary vascular involvement of igg4related disease. Pulmonary venoocclusive disease pvod is a rare entity that is usually mistaken with pulmonary arterial hypertension pah but is. For personal accounts or managers of institutional accounts. Histological features of a lung biopsy specimen from a 46yearold woman showed all the characteristics described in veno occlusive disease. Pulmonary venoocclusive disease pvod is defined by specific pathologic changes of the pulmonary veins. Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. Pulmonary capillary hemangiomatosis and pulmonary venoocclusive disease. Pulmonary venoocclusive disease and pulmonary hypertension in dogs. Click on the link to view a sample search on this topic. Pulmonary venoocclusive disease european respiratory society. A case of pulmonary venoocclusive disease respondong to. Hepatic venoocclusive disease vod, also called sinusoidal obstruction syndrome sos.
Pulmonary veno occlusive disease pvod is a rare cause of group 1 ph who group 1. At post mortem examination typical changes of pulmonary veno occlusive disease were found. The aim of this study was to describe our experience at one institution with pulmonary veno occlusive disease pvod during the past 10 years, with particular reference to new findings and long. The disease is progressive and fatal, with median survival of about 2 years from the time of diagnosis to death. However, ph remains an incurable condition with a high mortality rate, underscoring the need. Pulmonary venoocclusive disease pvod is characterized by the blockage occlusion of the blood vessels that carry oxygenrich oxygenated blood from the lungs to the heart the pulmonary veins. So far some 200 cases have been published worldwide. Pdf the authors sought to evaluate the diagnostic accuracy of highresolution computed tomography hrct in the detection of pulmonary.
First described in 1934 by dr julius hora of the university of munich, 1 pvod is characterized by widespread occlusion of the pulmonary venules by. Pulmonary veno occlusive disease pvod is a rare form of pulmonary hypertension ph characterised by preferential remodelling of the pulmonary venules. Pulmonary venoocclusive disease pvod is classified as a subgroup of pah and accounts for 510% of histological forms of cases initially considered to be idiopathic pah. It is a complication of highdose chemotherapy given before a bone marrow transplant bmt and is marked by weight gain due to fluid retention, increased liver size, and raised levels of bilirubin in the blood.
Pulmonary veno occlusive disease pvod is a rare and fatal cause of pulmonary hypertension ph that is difficult to diagnose and treat. Pulmonary veno occlusive disease after chemotherapy. The third patient underwent combination radiation and chemotherapy for hodgkins disease. Veno occlusive histopathologic changes were first described in 1934 as the cause of death in a 48yearold german baker with progressive dyspnea, cyanosis, and pulmonary edema, 1. The symptoms had not improved with inhaled corticosteroids fluticasone 250. Pulmonary venoocclusive disease in this setting is thought to be rare. Its aetiology is unknown, although it is sometimes associated with viral infection, environmental toxins, chemotherapy, autoimmune disease. Pulmonary veno occlusive disease pvod is a rare and devastating cause of pulmonary hypertension that is characterized histologically by widespread fibrous intimal proliferation of. Pulmonary veno occlusive disease pvod, lung, human. Pvod is a rare form of pah that remains poorly understood and is both difficult to diagnose and treat 611. Pulmonary veno occlusive disease pvod is a rare form of pulmonary hypertension ph. Pulmonary veno occlusive disease pvod is a rare and devastating cause of pulmonary arterial hypertension with a nonspecific clinical presentation and a relatively specific presentation in highresolution thoracic ct scan images. Endpoints of interest were overall survival os, progressionfree. Pulmonary venoocclusive disease and pulmonary capillary.
The aim of this study was to describe our experience at one institution with pulmonary veno occlusive disease pvod during the past 10 years, with particular reference to new findings and longterm outcome. Pulmonary capillary hemangiomatosis and pulmonary veno occlusive disease. Phase 3 trial of defibrotide for the treatment of severe veno occlusive disease and multiorgan failure. Editorials heritable pulmonary arterial hypertension idiopathic pulmonary arterial hypertension pulmonary veno occlusive disease. Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension characterised by predominant remodelling of pulmonary venules. Clinical and translational research has played a major role in advancing our understanding of pulmonary hypertension ph, including pulmonary arterial hypertension and other forms of ph with severe vascular remodelling e.
Pulmonary venoocclusive disease after chemotherapy. A case of pulmonary veno occlusive disease pvod in a 41yearold woman who had received chemotherapy bleomycin, mitomycinc, and cisplatinum for metastatic cervical carcinoma is reported. Defibrotide for patients with hepatic venoocclusive disease. Yasuhiro et al reported a case with igg4related lung obliterative phlebitis, with a hemodynamical change similar to that of pulmonary veno occlusive disease. Pulmonary venoocclusive disease pvod is a rare cause of pulmonary hypertension. Pulmonary arterial hypertension pah is a severe condition characterised by elevated pulmonary artery pressure leading to right heart failure and death 1, 2. Hepatic veno occlusive disease sinusoidal obstruction syndrome vodsos is a potentially lifethreatening complication of hematopoietic cell transplantation hct. Pulmonary venoocclusive disease is a rare and usually fatal condition in which there is gradual obliteration of the pulmonary veins and venules. Pulmonary venoocclusive disease and pulmonary hypertension in. Hepatic venoocclusive disease or veno occlusive disease with immunodeficiency vodi is a condition in which some of the small veins in the liver are obstructed. Sarcoidosisrelated pulmonary venoocclusive disease. Pulmonary veno occlusive disease pvod is a clinicopathologic syndrome that accounts for a small number of cases of pulmonary hypertension.
Pulmonary veno occlusive disease pvod is a rare condi tion in which the predominant anomaly is the stenosis or occlusion of the lumen of small pulmonary veins due to fibrosis of the intima 1. Eif2ak4 mutation in pulmonary venoocclusive disease. Definitive diagnosis is made by histological examination in previous. Biallelic mutations in the eukaryotic translation initiation factor 2. Pvodpch, the probability of eventfree survival, defined as death or. The occlusion is caused by a buildup of abnormal fibrous tissue in the small veins in the lungs, which narrows the vessels and impairs blood flow.
The patient denied fever, sweats or weight loss and had no significant past medical. 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. Related adverse events aes included hemorrhage or hypotension. Diagnosis and severity criteria for sinusoidal obstruction syndrome. Pulmonary venoocclusive disease is highly prevalent in. Eif2ak4 mutations cause pulmonary venoocclusive disease. Early clinical predictors of hepatic venoocclusive disease. The histological hallmark is obstructive intimal fibrosis of small veins and venules compared to the arteriolar pathology of. Sinusoidal obstruction syndrome or venoocclusive disease sosvod. The chest radiograph of the boy showed diffuse opacities, whereas that of the girl showed kerley lines bilaterally. Prophylactic, preemptive, and curative treatment for. Pulmonary veno occlusive disease pvod is a rare cause of pulmonary arterial hypertension pah in humans and can be classified in idiopathic, heritable, drug and radiationinduced, and associated with connective tissue disease or human immunodeficiency virus infection. The diagnosis of pulmonary veno occlusive disease and pulmonary capillary hemangiomatosis disease should be considered when ct findings of pulmonary hypertension are combined with centrilobular groundglass opacities, mediastinal lymph node enlargement, and smooth thickening of interlobular septa. Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension which.
Phase 3 trial of defibrotide for the treatment of severe. Pulmonary venoocclusive disease and the crest variant of. Hepatic sinusoidal obstruction syndromevenoocclusive disease sosvod is a lifethreatening complication of hct that belongs to a group of diseases. In both, at autopsy, a diagnosis of severe veno occlusive disease was confirmed, with partial or complete occlusion of much of the pulmonary venous bed by organized thrombi. In the current ph classification, pvod and pulmonary capillary haemangiomatosis pch are considered to be a common entity and represent varied expressions of the same disease. The clinical features, however, were distinctive in that in addition to the lung involvement there was alopecia, digital vasculitic ulcers, raynauds phenomenon, polyarthritis, and muscle weakness. She gave a 3yr history of dry cough, episodic wheeze and mild exertional dyspnoea. In 1966, british pathologist donald heath and associates, 2 were the first to assert that pulmonary veno occlusive disease pvod is a distinct entity rather than an incidental autopsy finding.
Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension ph characterised by preferential remodelling of the pulmonary venules. Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension ph. An unusual case presenting with thrombosis of the right pulmonary artery and. The term pulmonaryvaso occlusive disease has been suggested by others, asadditional pathologic evidence has demonstrated arterial as well asvenous changes.
Without a lung biopsy the clinical diagnosis of this disease is difficult. Manifestations and management of venoocclusive disease. Pulmonary venoocclusive disease pvod is currently classified as a subgroup of pulmonary arterial hypertension pah and accounts for 510% of cases initially considered to be idiopathic pah. Farber, md, the pulmonary center, boston university school of medicine, 72 east concord st, r304, boston, ma 02118.
Pulmonary veno occlusive disease is a rare and usually fatal condition in which there is gradual obliteration of the pulmonary veins. Hepatic venoocclusive disease vod, also known as sinusoidal obstruction. Pulmonary venoocclusive disease following therapy for. Pulmonary capillary hemangiomatosis and pulmonary veno. 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. It has a reported overall mean incidence of 814%, with an incidence of up to 60% in some highrisk populations 1, 2, 3. When caring for our patients with pulmonary arterial hypertension pah, how many times have we wondered, could this patient really have pulmonary veno occlusive disease pulmonary capillary hemangiomiatosis pvodpch. Pathology and pathobiology of pulmonary hypertension. Pulmonary venoocclusive disease pvod, a variant of pulmonary arterial hypertension, is a rare complication that can occur after hematopoietic stem cell transplantation hsct and chemotherapy. Recently, biallelic mutations of the eif2ak4 gene have been discovered as a cause for an autosomal recessive. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. Known causes of pvod include mutations in eif2ak4, reduced pulmonary vascular egrapelin signaling, and chemotherapy agents.
Sinusoidal obstruction syndromevenoocclusive disease. Pulmonary venoocclusive disease european respiratory. Pulmonary venoocclusive disease genetics home reference. Defibrotide has been approved for use in hsct patients with vodsos and renal or pulmonary dysfunction. Sinusoidal obstruction syndrome, also known as veno occlusive disease sosvod, is a potentially lifethreatening complication that can develop after hematopoietic cell transplantation hct. Pulmonary veno occlusive disease pvod is a rare form of pulmonary hypertension characterized by a progressive occlusion of the small pulmonary veins by fibrous tissue and intimal thickening. The concept of venoocclusive disease vod, along with our understanding of it, has historically. Pulmonary venoocclusive disease as a cause of severe. In humans, pulmonary venoocclusive disease pvod is a rare cause of severe pulmonary hypertension with a mean survival time of 2 years without lung transplantation. Pulmonary veno occlusive disease is classified as a subgroup of pulmonary arterial hypertension, with the observed postcapillary lesions of septal veins and preseptal venules consisting. Pulmonary venoocclusive disease american journal of. A 52yrold female nonsmoker presented with haemoptysis consisting of streaks of bright red blood daily for 3 weeks. Hepatic venoocclusive disease vod, or sinusoidal obstruction.
229 505 736 435 927 1170 1311 873 2 1011 1347 1279 157 11 1348 1 755 990 213 178 512 854 1529 1055 1221 363 1029 1070 541 504 1264 194 359 480