Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension.
Sarcoidosis Vasc Diffuse Lung Dis
; 33(2): 182-5, 2016 Aug 01.
Article
em En
| MEDLINE
| ID: mdl-27537724
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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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Sarcoidose Pulmonar
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Receptores de Proteínas Morfogenéticas Ósseas Tipo II
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Hipertensão Pulmonar Primária Familiar
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Mutação
Tipo de estudo:
Diagnostic_studies
/
Risk_factors_studies
Limite:
Adult
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Female
/
Humans
Idioma:
En
Revista:
Sarcoidosis Vasc Diffuse Lung Dis
Ano de publicação:
2016
Tipo de documento:
Article