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Title
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Extensive pulmonary sarcoid reaction in a patient with bmpr-2 associated idiopathic pulmonary arterial hypertension
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Author
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Abstract
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| 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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Language
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English
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Source (journal)
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Sarcoidosis vasculitis and diffuse lung disease. - Milano
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Publication
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Milano
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2016
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ISSN
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1124-0490
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Volume/pages
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33
:2
(2016)
, p. 182-185
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ISI
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000393272500014
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