Recently, a French group investigated the clinical and lung histology data for 24 PVOD patients (12 EIF2AK4 mutation carriers, 12 non-carriers), which reported that pulmonary artery remodeling and decreased GCN2 expression are the common denominators in all cases of PVOD, while the carriers of EIF2AK4 mutation presented a severe intimal fibrosis, stronger muscular hyperplasia of interlobular septal veins, less severe medial hypertrophy and decreased GCN2 expression than the non-carriers [7]. Here, EIF2AK4 is linked to pulmonary venoocclusive disease.