title: Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease.
abstract: This article provides an overview of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic pulmonary arterial hypertension (PAH). The article reviews the features that differentiate PVOD and PCH from PAH. The article also describes the overlap of PVOD and PCH, highlighted by recent reports of families diagnosed with PVOD or PCH caused by EIF2AK4 mutations. In addition, the article outlines current approaches to the diagnosis and treatment of PVOD and PCH.
authors: Respiratory Institute, Cleveland Clinic, Lerner College of Medicine of Case Western Reserve University, 9500 Euclid Avenue, A-90, Cleveland, OH 44195, USA.
authors: Neal F
authors: NF
authors: Chaisson
authors: Pulmonary Division, University of Utah, 24 North 1900 East Wintrobe Building, Room 701, Salt Lake City, UT 84132, USA; Department of Medicine, Intermountain Medical Center, 5121 South Cottonwood Street, Suite 307, Murray, UT 84107, USA.
authors: Mark W
authors: MW
authors: Dodson
authors: Pulmonary Division, University of Utah, 24 North 1900 East Wintrobe Building, Room 701, Salt Lake City, UT 84132, USA; Department of Medicine, Intermountain Medical Center, 5121 South Cottonwood Street, Suite 307, Murray, UT 84107, USA. Electronic address: greg.elliott@imail.org.
authors: Charles Gregory
authors: CG
authors: Elliott
mesh_terms: D003937:Diagnosis, Differential; D018324:Hemangioma, Capillary / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D009154:Mutation; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D017346:Protein Serine-Threonine Kinases / Q000235:genetics; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology
chemical_list: C529271:EIF2AK4 protein, human; D017346:Protein Serine-Threonine Kinases
keywords: Genetics; Pulmonary capillary hemangiomatosis; Pulmonary disorder; Pulmonary hypertension; Pulmonary veno-occlusive disease