Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture

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Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension characterised by extensive fibrotic occlusion of pulmonary veins. PVOD has a similar insidious presentation to idiopathic pulmonary arterial hypertension but responds poorly to conventional therapies and has a worse prognosis. The current study reports the case of a Caucasian female with a long history of progressive dyspnoea ultimately diagnosed as focal granulomatous venulitis leading to a pulmonary veno-occlusive disease-like pathology. The present study highlights the challenges in diagnosing and treating this condition. Copyright©ERS Journals Ltd 2009.

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Dwyer, N., Smith, R., Challis, D., Reid, D., & Kilpatrick, D. (2009). Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture. European Respiratory Journal, 33(3), 666–669. https://doi.org/10.1183/09031936.00001208

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