Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
CASE REPORTS
Lengthy Diagnostic Challenge in a Rare Case of Pulmonary Veno-Occlusive Disease: Case Report and Review of the Literature
Xiao-qun YeChun-song YanXin-yi ZhangYin CaiFeng GuoJiu-long Kuang
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JOURNALS OPEN ACCESS

2011 Volume 50 Issue 12 Pages 1323-1327

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Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare and usually survival poor disorder. We report a patient with a long history of progressive dyspnea of over 8 years, who with a diagnosis of chronic cor pulmonale confirmed elsewhere, was ultimately diagnosed as PVOD via histological analysis of a lung biopsy. After treatment with combined bosentan, diuretics and digoxin, his symptoms and function improved. This case highlights that PVOD is an under-recognised and often misdiagnosed disease, especially in its chronic form. Understanding its pathogenesis, its poor response to medical therapy and its dismal prognosis remain challenges for the treatment of PVOD.

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© 2011 by The Japanese Society of Internal Medicine
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