Progression of pulmonary veno‐occlusive disease without pulmonary hypertension
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 wi...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2022-01-01
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| Series: | Pulmonary Circulation |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/pul2.12046 |
| _version_ | 1811205883621801984 |
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| author | Sarah Beshay Marc Humbert Roberto Barrios Sandeep Sahay |
| author_facet | Sarah Beshay Marc Humbert Roberto Barrios Sandeep Sahay |
| author_sort | Sarah Beshay |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-12T03:38:54Z |
| format | Article |
| id | doaj.art-5f562114e5fa4b9abe771bb44a5b9e2e |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-04-12T03:38:54Z |
| publishDate | 2022-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-5f562114e5fa4b9abe771bb44a5b9e2e2022-12-22T03:49:20ZengWileyPulmonary Circulation2045-89402022-01-01121n/an/a10.1002/pul2.12046Progression of pulmonary veno‐occlusive disease without pulmonary hypertensionSarah Beshay0Marc Humbert1Roberto Barrios2Sandeep Sahay3Department of Internal Medicine, Division of Pulmonary, Critical Care & Sleep Medicine Houston Methodist Lung Center Houston Texas USASchool of Medicine Université Paris‐Saclay Le Kremlin‐Bicêtre FranceDepartment of Pathology and Genomics Houston Methodist Hospital Houston Texas USADepartment of Internal Medicine, Division of Pulmonary, Critical Care & Sleep Medicine Houston Methodist Lung Center Houston Texas USAAbstract 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.https://doi.org/10.1002/pul2.12046drug toxicitypulmonary hypertensionpulmonary veno‐occlusive disease |
| spellingShingle | Sarah Beshay Marc Humbert Roberto Barrios Sandeep Sahay Progression of pulmonary veno‐occlusive disease without pulmonary hypertension Pulmonary Circulation drug toxicity pulmonary hypertension pulmonary veno‐occlusive disease |
| title | Progression of pulmonary veno‐occlusive disease without pulmonary hypertension |
| title_full | Progression of pulmonary veno‐occlusive disease without pulmonary hypertension |
| title_fullStr | Progression of pulmonary veno‐occlusive disease without pulmonary hypertension |
| title_full_unstemmed | Progression of pulmonary veno‐occlusive disease without pulmonary hypertension |
| title_short | Progression of pulmonary veno‐occlusive disease without pulmonary hypertension |
| title_sort | progression of pulmonary veno occlusive disease without pulmonary hypertension |
| topic | drug toxicity pulmonary hypertension pulmonary veno‐occlusive disease |
| url | https://doi.org/10.1002/pul2.12046 |
| work_keys_str_mv | AT sarahbeshay progressionofpulmonaryvenoocclusivediseasewithoutpulmonaryhypertension AT marchumbert progressionofpulmonaryvenoocclusivediseasewithoutpulmonaryhypertension AT robertobarrios progressionofpulmonaryvenoocclusivediseasewithoutpulmonaryhypertension AT sandeepsahay progressionofpulmonaryvenoocclusivediseasewithoutpulmonaryhypertension |