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

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.