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. 182-5
Article / Letter to editor
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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.
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