Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension
SourceSarcoidosis, Vasculitis, and Diffuse Lung Diseases, 33, 2, (2016), pp. 182-185
Article / Letter to editor
Display more detailsDisplay less details
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases
SubjectRadboudumc 5: Inflammatory diseases RIHS: Radboud Institute for Health Sciences
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.
Upload full text
Use your RU credentials (u/z-number and password) tolog in with SURFconextto upload a file for processing by the repository team.