title: Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension is a progressive life-threatening disease characterized by vascular remodeling. There is evidence that varied immune mechanism play an important role in progression of pulmonary hypertension.  We describe a case of a 35-year-old woman with idiopathic pulmonary arterial hypertension (IPAH) and a novel BMPR2 mutation, who underwent a successful lung transplantation.  Extensive granulomatous inflammation was seen in the resected lungs. The granulomatous inflammation found in the histology supports  a sarcoid-like reaction due to pulmonary hypertension in the context of the BMPR2 mutation.
authors.affiliation: Radboud University Medical Center. Evelien.Braam@radboudumc.nl.
authors.forename: Evelien A J E
authors.initials: EA
authors.lastname: Braam
authors.forename: Marian J R
authors.initials: MJ
authors.lastname: Quanjel
authors.forename: Jolanda H G M
authors.initials: JH
authors.lastname: Van Haren-Willems
authors.forename: Matthijs F M
authors.initials: MF
authors.lastname: Van Oosterhout
authors.forename: Aryan
authors.initials: A
authors.lastname: Vink
authors.forename: Yvonne F
authors.initials: YF
authors.lastname: Heijdra
authors.forename: Johanna M
authors.initials: JM
authors.lastname: Kwakkel-van Erp
mesh_terms: D000328:Adult; D001706:Biopsy; D052006:Bone Morphogenetic Protein Receptors, Type II / Q000235:genetics*; D065627:Familial Primary Pulmonary Hypertension / Q000150:complications / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D016040:Lung Transplantation; D009154:Mutation; D017565:Sarcoidosis, Pulmonary / Q000175:diagnosis / Q000209:etiology*
chemical_list: C498218:BMPR2 protein, human; D052006:Bone Morphogenetic Protein Receptors, Type II
keywords: Granulomatosis; Lung transplant.; Pulmonary arterial hypertension; Sarcoidosis