Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension

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Evelien A.J.E. Braam
Marian J.R. Quanjel
Jolanda H.G.M. van Haren-Willems
Matthijs F.M. van Oosterhout
Aryan Vink
Yvonne F. Heijdra
Johanna M. Kwakkel-van Erp

Keywords

Pulmonary arterial hypertension, Granulomatosis, Sarcoidosis, Lung transplant.

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. 

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