肺静脉闭塞性疾病的诊断和治疗。

IF 2.9 3区 医学 Q2 RESPIRATORY SYSTEM Expert Review of Respiratory Medicine Pub Date : 2023-07-01 Epub Date: 2023-08-21 DOI:10.1080/17476348.2023.2247989
Sabina Solinas, Athénaïs Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Mélanie Eyries, Peter Dorfmüller, Olivier Sitbon, Marc Humbert, David Montani
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引用次数: 0

摘要

引言:肺静脉闭塞性疾病(PVOD)是一种孤立性疾病,也是肺动脉高压(PAH)的罕见病因,其特征是大量小肺静脉和毛细血管受累。涵盖的领域:PVOD,在当前ESC/ERS分类中也称为“具有静脉/毛细血管受累特征的PAH”。专家意见:近年来,PVOD的特定风险因素已被认识到,包括遗传易感性和环境因素(如接触职业有机溶剂、化疗和潜在的烟草)。在EIF2AK4基因中发现双等位基因突变是可遗传PVOD的原因,这是理解PVOD分子基础的突破。据报道,静脉和毛细血管受累(PVOD样)在结缔组织疾病相关的PAH(尤其是系统性硬化症)以及结节病和肺郎格汉斯细胞肉芽肿等罕见肺部疾病中也相对常见。尽管PVOD和肺动脉高压(PAH)表现出相似之处,包括严重的毛细血管前PH,但有必要区分它们,因为PVOD的预后较差,需要特殊的治疗。事实上,PVOD患者的特点是对PAH批准的药物反应不佳,这可能导致肺水肿和临床恶化。由于缺乏有效的治疗方法,尽早转诊到肺移植中心至关重要。
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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.

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来源期刊
CiteScore
6.80
自引率
2.60%
发文量
90
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
期刊最新文献
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