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Romanian Journal of Internal Medicine

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Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in systemic sclerosis. Case presentation and review of the literature

Ana Maria Daraban
  • Clinical Emergency Hospital Bucharest, Romania
  • “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
/ Roxana Enache
  • “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
/ L. Predescu
  • “C.C. Iliescu” Emergency Institute for Cardiovascular Diseases, Bucharest, Romania
/ P. Platon
  • “C.C. Iliescu” Emergency Institute for Cardiovascular Diseases, Bucharest, Romania
/ T. Constantinescu
  • “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
  • “Prof. Dr. Marius Nasta” Pneumology Institute, Bucharest, Romania
/ Carina Mihai
  • “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
  • “Cantacuzino” Clinical Hospital, Bucharest, Romania
/ I.M. Coman
  • “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
  • “C.C. Iliescu” Emergency Institute for Cardiovascular Diseases, Bucharest, Romania
/ Carmen Ginghină
  • “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
  • “C.C. Iliescu” Emergency Institute for Cardiovascular Diseases, Bucharest, Romania
/ Ruxandra Jurcuţ
  • Corresponding author
  • “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
  • “C.C. Iliescu” Emergency Institute for Cardiovascular Diseases, Bucharest, Romania
  • Email:
Published Online: 2015-10-14 | DOI: https://doi.org/10.1515/rjim-2015-0024

Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH). Because of the similar clinical picture of dyspnea on exertion and signs of right heart failure, PVOD is difficult to distinguish from idiopathic PAH. However, the distinction is mandatory because PVOD has a worse prognosis and, more importantly, the administration of PAH specific therapy (vasodilators) can precipitate severe acute pulmonary oedema. We present a challenging case of PAH in a patient with systemic sclerosis in whom a marked decrease in functional capacity after the initiation of bosentan therapy led to the diagnosis of PVOD. Management of PVOD patients is challenging and referral for lung transplantation should be done at the moment of diagnosis.

Boala pulmonară veno-ocluzivă (BPVO) este o cauză rară de hipertensiune arterială pulmonară (HTP). Datorită aspectului similar clinic îmbrăcat de BPVO şi de HTP idiopatică caracterizate prin dispnee de efort şi semne ale insuficienţei cardiace drepte, cele două entităţi sunt dificil de diferenţiat. Totuşi, distincţia dintre cele două este obligatorie deoarece BPVO prezintă un prognostic mai prost decât HTP idiopatică şi, mai important, administrarea terapiei specifice pentru HTP (vasodilatatoare) poate precipita edemul pulmonar acut sever. Este prezentat un caz interesant al unei paciente diagnosticată cu sclerodermie sistemică la care diagnosticul de BPVO a fost realizat după ce capacitatea sa pulmonară s-a degradat după iniţierea terapiei cu bosentan. Managementul pacienţilor cu BPVO reprezintă o provocare şi trimiterea către transplant pulmonar ar trebui realizată odată cu punerea diagnosticului.

Keywords: Pulmonary arterial hypertension; pulmonary venoocclusive disease; Systemic sclerosis

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About the article

Published Online: 2015-10-14

Published in Print: 2015-06-01



Citation Information: Romanian Journal Of Internal Medicine, ISSN (Online) 1220-4749, DOI: https://doi.org/10.1515/rjim-2015-0024. Export Citation

© 2015 Ana Maria Daraban et al., published by De Gruyter Open. This chapter is distributed under the terms of the Creative Commons Attribution 4.0 Public License. (CC BY 4.0)

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