Progression of pulmonary veno‐occlusive disease without pulmonary hypertension. (21st February 2022)

• Record Type: Journal Article
• Authors: Beshay, Sarah; Humbert, Marc; Barrios, Roberto; Sahay, Sandeep
• Is Part Of: Pulmonary circulation. Volume 12:Number 1(2022)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2022-02-21
• Subjects: drug toxicity -- pulmonary hypertension -- pulmonary veno‐occlusive disease; Pulmonary circulation -- Periodicals; Pulmonary circulation; Electronic journals -- Sciences; Periodicals; 616.24005
• DOI: 10.1002/pul2.12046
• Languages: English
• ISSNs: 2045-8932

Abstract

Abstract: 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.