Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension
Publication date
2016-08-01
Authors
Braam, Evelien A J E
Quanjel, Marian J.R.
Van Haren-Willems, Jolanda H G M
Van Oosterhout, Matthijs F M
Vink, A.
Heijdra, Yvonne F
Kwakkel-van Erp, J. M.
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DOI
Document Type
Article
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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.
Keywords
Pulmonary arterial hypertension, Granulomatosis, Sarcoidosis, Lung transplant
Citation
Braam, E A J E, Quanjel, M J R, Van Haren-Willems, J H G M, Van Oosterhout, M F M, Vink, A, Heijdra, Y F & Kwakkel-van Erp, J M 2016, 'Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension', Sarcoidosis, Vasculitis, and Diffuse Lung Diseases, vol. 33, no. 2, pp. 182-185. < http://www.mattioli1885journals.com/index.php/sarcoidosis/article/view/4592 >