title: Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture.
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.
authors.affiliation: Cardiology Department, Royal Hobart Hospital, 48 Liverpool St, Hobart, Tasmania, Australia. ndwyer@utas.edu.au
authors.forename: N
authors.initials: N
authors.lastname: Dwyer
authors.forename: R
authors.initials: R
authors.lastname: Smith
authors.forename: D
authors.initials: D
authors.lastname: Challis
authors.forename: D
authors.initials: D
authors.lastname: Reid
authors.forename: D
authors.initials: D
authors.lastname: Kilpatrick
mesh_terms: D000328:Adult; D001706:Biopsy; D004487:Edema; D017809:Fatal Outcome; D005260:Female; D005355:Fibrosis; D006439:Hemodynamics; D006801:Humans; D009336:Necrosis; D011379:Prognosis; D011667:Pulmonary Veins / Q000503:physiopathology; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000175:diagnosis*; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis*