Pulmonary veno-occlusive disease

被引：7
|
作者：
Boucly, A. [1 ,2 ,3 ]
Girerd, B. [1 ,2 ,3 ]
Bourlier, D. [1 ,2 ,3 ]
Nemlaghi, S. [1 ,2 ,3 ]
Caliez, J. [1 ,2 ,3 ]
Savale, L. [1 ,2 ,3 ]
Jais, X. [1 ,2 ,3 ]
Dorfmueller, P. [1 ,2 ,4 ]
Simonneau, G. [1 ,2 ,3 ]
Sitbon, O. [1 ,2 ,3 ]
Humbert, M. [1 ,2 ,3 ]
Montani, D. [1 ,2 ,3 ]
机构：
[1] Univ Paris Sud, Fac Med, F-94270 Le Kremlin Bicetre, France
[2] Univ Paris Sud, Hop Bicetre, AP HP,Ctr Reference Hypertens Pulm Severe, Dept Hosp Univ DHU Thorax Innovat TORINO,Serv Pne, 78 Rue Gen Leclerc, F-94270 Le Kremlin Bicetre, France
[3] Univ Paris Sud, UMR S999, INSERM, Hop Marie Lannelongue,Lab Excellence LabEx Rech M, F-92350 Le Plessis Robinson, France
[4] Ctr Chirurg Marie Lannelongue, Serv Anatomopathol, F-92350 Le Plessis Robinson, France
来源：
REVUE DES MALADIES RESPIRATOIRES | 2018年 / 35卷 / 02期
关键词：
Pulmonary veno-occlusive disease; Diagnosis; Therapy; Prognosis; ARTERIAL-HYPERTENSION; KINASE GCN2; PROSTACYCLIN; THERAPY; PATIENT; EXPOSURE;
D O I：
10.1016/j.rmr.2017.11.005
中图分类号：
R56 [呼吸系及胸部疾病];
学科分类号：
摘要：
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venutes and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding. A noninvasive diagnostic approach, including oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest, is used to support a diagnosis of PVOD. PVOD prognosis is worse than other forms of PAH. There is no evidence-based medical therapy for PVOD and life-threatening pulmonary edema may occur following PAH targeted therapy in PVOD. Lung transplantation remains the preferred definitive therapy for eligible patients. (C) 2017 SPLF. Published by Elsevier Masson SAS. All rights reserved.
引用
收藏
页码：160 / 170
页数：11
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