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

¹ Depts of Cardiology, ² Anatomical Pathology, and ³ Respiratory Medicine, Royal Hobart Hospital, Tasmania, Australia.

CORRESPONDENCE: N. Dwyer, Cardiology Dept, Royal Hobart Hospital, 48 Liverpool St, Hobart, Tasmania, Australia 7000. Fax: 61 62342852. E-mail: ndwyer{at}utas.edu.au

Keywords: Acute pulmonary oedema, bosentan, granulomatous venulitis, infliximab, pulmonary veno-occlusive disease, sildenafil

Received: January 3, 2008
Accepted September 4, 2008

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.