Progression of pulmonary veno-occlusive disease without pulmonary hypertension

Sarah Beshay, Marc Humbert, Roberto Barrios, Sandeep Sahay

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


Pulmonary veno-occlusive disease (PVOD) is a progressively fatal disease with no definitive treatment options. PVOD can be a result of genetic mutation but can also be due secondary to exposure to solvents or chemotherapeutic agents. Generally, at the time of diagnosis PVOD is associated with hemodynamically confirmed pulmonary hypertension (PH). In this study, we describe a patient who was diagnosed with PVOD early in the disease without hemodynamically confirmed PH. She had histologically confirmed PVOD. Her clinical presentation posed management challenges and prednisone therapy was used to stabilize her disease. This case and some recently published reports highlight possible immune dysregulation in PVOD and role for immuno-suppressive therapy in these patients.

Original languageEnglish (US)
Article numbere12046
Pages (from-to)e12046
JournalPulmonary Circulation
Issue number1
StatePublished - Jan 2022


  • drug toxicity
  • pulmonary hypertension
  • pulmonary veno-occlusive disease

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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