[Pulmonary veno-occlusive disease]

A Boucly; B Girerd; D Bourlier; S Nemlaghi; J Caliez; L Savale; X Jaïs; P Dorfmüller; G Simonneau; O Sitbon; M Humbert; D Montani

doi:10.1016/j.rmr.2017.11.005

[Pulmonary veno-occlusive disease]

Rev Mal Respir. 2018 Feb;35(2):160-170. doi: 10.1016/j.rmr.2017.11.005. Epub 2018 Mar 1.

[Article in French]

Authors

A Boucly¹, B Girerd¹, D Bourlier¹, S Nemlaghi¹, J Caliez¹, L Savale¹, X Jaïs¹, P Dorfmüller², G Simonneau¹, O Sitbon¹, M Humbert¹, D Montani³

Affiliations

¹ Faculté de médecine, university Paris-Sud, 94270 Le Kremlin-Bicêtre, France; Département hospitalo-universitaire (DHU) thorax innovation (TORINO), service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, hôpital de Bicêtre, université Paris-Sud, AP-HP, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; UMR_S999, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), hôpital Marie-Lannelongue, university Paris-Sud, 92350 Le Plessis-Robinson, France.
² Faculté de médecine, university Paris-Sud, 94270 Le Kremlin-Bicêtre, France; Département hospitalo-universitaire (DHU) thorax innovation (TORINO), service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, hôpital de Bicêtre, université Paris-Sud, AP-HP, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; Service d'anatomopathologie, centre chirurgical Marie-Lannelongue, 92350 Le Plessis Robinson, France.
³ Faculté de médecine, university Paris-Sud, 94270 Le Kremlin-Bicêtre, France; Département hospitalo-universitaire (DHU) thorax innovation (TORINO), service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, hôpital de Bicêtre, université Paris-Sud, AP-HP, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; UMR_S999, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), hôpital Marie-Lannelongue, university Paris-Sud, 92350 Le Plessis-Robinson, France. Electronic address: [email protected].

PMID: 29501213
DOI: 10.1016/j.rmr.2017.11.005

Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules 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.

Keywords: Diagnosis; Diagnostic; Maladie veino-occlusive pulmonaire; Prognosis; Pronostic; Pulmonary veno-occlusive disease; Therapy; Traitement.

Publication types

Review

MeSH terms

Animals
Diagnostic Imaging / methods
Disease Models, Animal
Humans
Pulmonary Veno-Occlusive Disease* / diagnosis
Pulmonary Veno-Occlusive Disease* / epidemiology
Pulmonary Veno-Occlusive Disease* / therapy
Rare Diseases / diagnosis
Rare Diseases / epidemiology
Rare Diseases / therapy
Respiratory Function Tests / methods
Risk Factors