Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture.
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.
Dwyer
N
N
Cardiology Department, Royal Hobart Hospital, 48 Liverpool St, Hobart, Tasmania, Australia. ndwyer@utas.edu.au
Smith
R
R
Challis
D
D
Reid
D
D
Kilpatrick
D
D
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*