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

Authors
  • Dwyer, N1
  • Smith, R
  • Challis, D
  • Reid, D
  • Kilpatrick, D
  • 1 Cardiology Department, Royal Hobart Hospital, 48 Liverpool St, Hobart, Tasmania, Australia. [email protected] , (Australia)
Type
Published Article
Journal
European Respiratory Journal
Publisher
European Respiratory Society
Publication Date
Mar 01, 2009
Volume
33
Issue
3
Pages
666–669
Identifiers
DOI: 10.1183/09031936.00001208
PMID: 19251802
Source
Medline
License
Unknown

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.

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