pulmonary venoocclusive disease 2 (DOID:0081269)
Alliance: disease page
Synonyms: FAMILIAL PULMONARY CAPILLARY HEMANGIOMATOSIS
Alt IDs: OMIM:234810
Definition: A pulmonary venoocclusive disease that has_material_basis_in homozygous or compound heterozygous mutation in the EIF2AK4 gene on chromosome 15q15 and that is characterized histologically by widespread fibrous intimal proliferation of septal veins and preseptal venules, and is frequently associated with pulmonary capillary dilatation and proliferation.