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

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

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

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

Research output: Contribution to journal › Article › Academic › peer-review

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.

Original language	English
Pages (from-to)	182-185
Number of pages	4
Journal	Sarcoidosis, Vasculitis, and Diffuse Lung Diseases
Volume	33
Issue number	2
Publication status	Published - 1 Aug 2016

Keywords

Pulmonary arterial hypertension
Granulomatosis
Sarcoidosis
Lung transplant

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4592-15634-2-PBFinal published version, 3.82 MBLicence: CC BY-NC

http://www.mattioli1885journals.com/index.php/sarcoidosis/article/view/4592Licence: CC BY-NC

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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, 33(2), 182-185. http://www.mattioli1885journals.com/index.php/sarcoidosis/article/view/4592

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title = "Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension",

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",

author = "Braam, {Evelien A J E} and Quanjel, {Marian J R} and {Van Haren-Willems}, {Jolanda H G M} and {Van Oosterhout}, {Matthijs F M} and Aryan Vink and Heijdra, {Yvonne F} and {Kwakkel-van Erp}, {Johanna M}",

year = "2016",

month = aug,

day = "1",

language = "English",

volume = "33",

pages = "182--185",

journal = "Sarcoidosis, Vasculitis, and Diffuse Lung Diseases",

issn = "1124-0490",

publisher = "Mattioli 1885 S.p.A.",

number = "2",

}

Braam, EAJE, Quanjel, MJR, Van Haren-Willems, JHGM, Van Oosterhout, MFM, Vink, A, Heijdra, YF & Kwakkel-van Erp, JM 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>

Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension. / Braam, Evelien A J E; Quanjel, Marian J R; Van Haren-Willems, Jolanda H G M et al.
In: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases, Vol. 33, No. 2, 01.08.2016, p. 182-185.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension

AU - Braam, Evelien A J E

AU - Quanjel, Marian J R

AU - Van Haren-Willems, Jolanda H G M

AU - Van Oosterhout, Matthijs F M

AU - Vink, Aryan

AU - Heijdra, Yvonne F

AU - Kwakkel-van Erp, Johanna M

PY - 2016/8/1

Y1 - 2016/8/1

N2 - 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.

AB - 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.

KW - Pulmonary arterial hypertension

KW - Granulomatosis

KW - Sarcoidosis

KW - Lung transplant

M3 - Article

C2 - 27537724

SN - 1124-0490

VL - 33

SP - 182

EP - 185

JO - Sarcoidosis, Vasculitis, and Diffuse Lung Diseases

JF - Sarcoidosis, Vasculitis, and Diffuse Lung Diseases

IS - 2

ER -

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

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Keywords

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