PT - JOURNAL ARTICLE AU - David Montani AU - Edmund M. Lau AU - Alexis Descatha AU - Xavier Jaïs AU - Laurent Savale AU - Pascal Andujar AU - Lynda Bensefa-Colas AU - Barbara Girerd AU - Inès Zendah AU - Jerome Le Pavec AU - Andrei Seferian AU - Frédéric Perros AU - Peter Dorfmüller AU - Elie Fadel AU - Florent Soubrier AU - Oliver Sitbon AU - Gérald Simonneau AU - Marc Humbert TI - Occupational exposure to organic solvents: a risk factor for pulmonary veno-occlusive disease AID - 10.1183/13993003.00814-2015 DP - 2015 Dec 01 TA - European Respiratory Journal PG - 1721--1731 VI - 46 IP - 6 4099 - http://erj.ersjournals.com/content/46/6/1721.short 4100 - http://erj.ersjournals.com/content/46/6/1721.full SO - Eur Respir J2015 Dec 01; 46 AB - Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension characterised by predominant remodelling of pulmonary venules. Bi-allelic mutations in the eukaryotic translation initiation factor 2α kinase 4 (EIF2AK4) gene were recently described as the major cause of heritable PVOD, but risk factors associated with PVOD remain poorly understood. Occupational exposures have been proposed as a potential risk factor for PVOD, but epidemiological studies are lacking.A case–control study was conducted in consecutive PVOD (cases, n=33) and pulmonary arterial hypertension patients (controls, n=65). Occupational exposure was evaluated via questionnaire interview with blinded assessments using an expert consensus approach and a job exposure matrix (JEM).Using the expert consensus approach, PVOD was significantly associated with occupational exposure to organic solvents (adjusted OR 12.8, 95% CI 2.7–60.8), with trichloroethylene being the main agent implicated (adjusted OR 8.2, 95% CI 1.4–49.4). JEM analysis independently confirmed the association between PVOD and trichloroethylene exposure. Absence of significant trichloroethylene exposure was associated with a younger age of disease (54.8±21.4 years, p=0.037) and a high prevalence of harbouring bi-allelic EIF2AK4 mutations (41.7% versus 0%, p=0.015).Occupational exposure to organic solvents may represent a novel risk factor for PVOD. Genetic background and environmental exposure appear to influence the phenotypic expression of the disease.Occupational exposure to organic solvents is a novel risk factor for pulmonary veno-occlusive disease http://ow.ly/StFsk