Sabina Solinas, Athénaïs Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Mélanie Eyries, Peter Dorfmüller, Olivier Sitbon, Marc Humbert, David Montani
{"title":"肺静脉闭塞性疾病的诊断和治疗。","authors":"Sabina Solinas, Athénaïs Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Mélanie Eyries, Peter Dorfmüller, Olivier Sitbon, Marc Humbert, David Montani","doi":"10.1080/17476348.2023.2247989","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Pulmonary veno-occlusive disease (PVOD) is an orphan disease and uncommon etiology of pulmonary arterial hypertension (PAH) characterized by substantial small pulmonary vein and capillary involvement.</p><p><strong>Areas covered: </strong>PVOD, also known as 'PAH with features of venous/capillary involvement' in the current ESC/ERS classification.</p><p><strong>Expert opinion: </strong>In recent years, particular risk factors for PVOD have been recognized, including genetic susceptibilities and environmental factors (such as exposure to occupational organic solvents, chemotherapy, and potentially tobacco). The discovery of biallelic mutations in the EIF2AK4 gene as the cause of heritable PVOD has been a breakthrough in understanding the molecular basis of PVOD. Venous and capillary involvement (PVOD-like) has also been reported to be relatively common in connective tissue disease-associated PAH (especially systemic sclerosis), and in rare pulmonary diseases like sarcoidosis and pulmonary Langerhans cell granulomatosis. Although PVOD and pulmonary arterial hypertension (PAH) exhibit similarities, including severe precapillary PH, it is essential to differentiate between them since PVOD has a worse prognosis and requires specific management. Indeed, PVOD patients are characterized by poor response to PAH-approved drugs, which can lead to pulmonary edema and clinical deterioration. Due to the lack of effective treatments, early referral to a lung transplantation center is crucial.</p>","PeriodicalId":12103,"journal":{"name":"Expert Review of Respiratory Medicine","volume":null,"pages":null},"PeriodicalIF":2.9000,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Diagnosis and management of pulmonary veno-occlusive disease.\",\"authors\":\"Sabina Solinas, Athénaïs Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Mélanie Eyries, Peter Dorfmüller, Olivier Sitbon, Marc Humbert, David Montani\",\"doi\":\"10.1080/17476348.2023.2247989\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Pulmonary veno-occlusive disease (PVOD) is an orphan disease and uncommon etiology of pulmonary arterial hypertension (PAH) characterized by substantial small pulmonary vein and capillary involvement.</p><p><strong>Areas covered: </strong>PVOD, also known as 'PAH with features of venous/capillary involvement' in the current ESC/ERS classification.</p><p><strong>Expert opinion: </strong>In recent years, particular risk factors for PVOD have been recognized, including genetic susceptibilities and environmental factors (such as exposure to occupational organic solvents, chemotherapy, and potentially tobacco). The discovery of biallelic mutations in the EIF2AK4 gene as the cause of heritable PVOD has been a breakthrough in understanding the molecular basis of PVOD. Venous and capillary involvement (PVOD-like) has also been reported to be relatively common in connective tissue disease-associated PAH (especially systemic sclerosis), and in rare pulmonary diseases like sarcoidosis and pulmonary Langerhans cell granulomatosis. Although PVOD and pulmonary arterial hypertension (PAH) exhibit similarities, including severe precapillary PH, it is essential to differentiate between them since PVOD has a worse prognosis and requires specific management. Indeed, PVOD patients are characterized by poor response to PAH-approved drugs, which can lead to pulmonary edema and clinical deterioration. 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Diagnosis and management of pulmonary veno-occlusive disease.
Introduction: Pulmonary veno-occlusive disease (PVOD) is an orphan disease and uncommon etiology of pulmonary arterial hypertension (PAH) characterized by substantial small pulmonary vein and capillary involvement.
Areas covered: PVOD, also known as 'PAH with features of venous/capillary involvement' in the current ESC/ERS classification.
Expert opinion: In recent years, particular risk factors for PVOD have been recognized, including genetic susceptibilities and environmental factors (such as exposure to occupational organic solvents, chemotherapy, and potentially tobacco). The discovery of biallelic mutations in the EIF2AK4 gene as the cause of heritable PVOD has been a breakthrough in understanding the molecular basis of PVOD. Venous and capillary involvement (PVOD-like) has also been reported to be relatively common in connective tissue disease-associated PAH (especially systemic sclerosis), and in rare pulmonary diseases like sarcoidosis and pulmonary Langerhans cell granulomatosis. Although PVOD and pulmonary arterial hypertension (PAH) exhibit similarities, including severe precapillary PH, it is essential to differentiate between them since PVOD has a worse prognosis and requires specific management. Indeed, PVOD patients are characterized by poor response to PAH-approved drugs, which can lead to pulmonary edema and clinical deterioration. Due to the lack of effective treatments, early referral to a lung transplantation center is crucial.
期刊介绍:
Coverage will include the following key areas:
- Prospects for new and emerging therapeutics
- Epidemiology of disease
- Preventive strategies
- All aspects of COPD, from patient self-management to systemic effects of the disease and comorbidities
- Improved diagnostic methods, including imaging techniques, biomarkers and physiological tests.
- Advances in the treatment of respiratory infections and drug resistance issues
- Occupational and environmental factors
- Progress in smoking intervention and cessation methods
- Disease and treatment issues for defined populations, such as children and the elderly
- Respiratory intensive and critical care
- Updates on the status and advances of specific disease areas, including asthma, HIV/AIDS-related disease, cystic fibrosis, COPD and sleep-disordered breathing morbidity