PULMONARY VENO OCCLUSIVE DISEASE- A RARE CAUSE OF PULMONARY ARTERIAL HYPERTENSION

Pulmonary veno occlusive disease (PVOD) is a rare cause of pulmonary arterialhypertension(PAH). The pathologic hallmark of PVOD is extensive and diffuse occlusion of thepulmonary veins by fibrous tissue. Most patients have nonspecific complaints and the clinical signs closely resemble idiopathic PAH. There are no definitive clinical criteria for the diagnosis of PAH and surgical lung biopsy is the current gold standard for diagnosis. Therapeutic options include long term oxygen therapy, oral anticoagulation with a target INR of 2 to 3, vasodilators and immunosuppressive therapy in case of association with autoimmune disease. The efficacy of current therapeutic options in not established owing to the rare nature of the disease and lack of randomized control trials. The prognosis of PVOD is poor and most patients die within two years of diagnosis.