pulmonary veno-occlusive disease


Also found in: Acronyms.

pulmonary veno-occlusive disease

An extremely rare idiopathic condition that may affect children and young adults, which causes progressive fibrous obliteration of veins, resulting in severe postcapillary pulmonary hypertension.
 
Aetiology
Uncertain, possibly linked to bone marrow transplants, chemotherapy (e.g., carmustine), malignancy (especially lymphoproliferative—e.g., lymphoma), leukaemia, viral infection, systemic lupus erythematosus.
 
Clinical findings
Dyspnoea, exertional fatigue, syncope, haemoptysis.

Imaging
Prominent interstitial markings, Kerley B lines, pulmonary arterial dilatation.

Management
Temporary response with vasodilators, nifedipine, alpha-adrenergic blocker, prazosin, and immunosuppressants; definitive therapy requires lung transplantation.
 
Prognosis
Poor—most die in 2 years.

pulmonary veno-occlusive disease

An idiopathic condition of children/young adults that causes progressive fibrous obliteration of veins, resulting in severe postcapillary pulmonary hypertension Etiology Uncertain, possibly linked to BM transplantation, chemotherapy–eg, carmustine, malignancy, viral infection Clinical Nodular zones of congestion, edema, hemorrhage, hemosiderosis Radiology CXR reveal prominent interstitial markings, Kerley B lines, pulmonary artery dilatation Management Temporary response may occur with vasodilators, nifedipine, α-adrenergic blocker, prazosin Prognosis Poor, most die in 2 yrs

pulmonary veno-occlusive disease

A condition that may complicate organ transplantation rejection. It is marked by extensive occlusion of the small and medium-sized veins of the lung by loose, sparsely cellular, fibrous tissue. Some larger veins may be involved. This disease produces severe pulmonary venous hypertension.
References in periodicals archive ?
(28) Interestingly, BMPR2 mutations are rarely detected in familial cases of pulmonary veno-occlusive diseases. (29) Instead, both PVOD and PCH share autosomal recessive mutations in the eukaryotic translation initiation factor 2 [alpha]-kinase 4 (EIF2AK4) gene and lead to proliferation of capillaries in the lung.
Pulmonary veno-occlusive disease (PVOD) and/o pulmonary capillary hemangiomatosis (PCH) 2.
Pulmonary veno-occlusive disease. Thorax 1971; 26: 663-74.
Pulmonary veno-occlusive disease: entity or syndrome?
Pulmonary veno-occlusive disease: Recent progress and current challenges.
Pulmonary veno-occlusive disease. Eur Respir J 2009; 33:189-200.
Pulmonary veno-occlusive disease in a HIV-infected intravenous drug abuser.
Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II.
Pulmonary veno-occlusive disease in a patient with a history of Hashimoto's thyroiditis.

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