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Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease characterized by the remodelling of distal pulmonary arteries in the absence of other cardiopulmonary disease, usually leading to right ventricular failure. Given the current European Society of Cardiol...

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Autores principales: Tanaka, Toshikazu D, Misawa, Sonoko, Yoshimura, Michihiro, Kuwabara, Satoshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6177100/
https://www.ncbi.nlm.nih.gov/pubmed/31020130
http://dx.doi.org/10.1093/ehjcr/yty051
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author Tanaka, Toshikazu D
Misawa, Sonoko
Yoshimura, Michihiro
Kuwabara, Satoshi
author_facet Tanaka, Toshikazu D
Misawa, Sonoko
Yoshimura, Michihiro
Kuwabara, Satoshi
author_sort Tanaka, Toshikazu D
collection PubMed
description INTRODUCTION: Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease characterized by the remodelling of distal pulmonary arteries in the absence of other cardiopulmonary disease, usually leading to right ventricular failure. Given the current European Society of Cardiology and the European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension (PH), most of the patients with severe PAH usually require to have a lifelong combination therapy that includes prostacyclin, phosphodiesterase-5 inhibitor, and endothelin receptor antagonist. However, the reversibility of PAH has been reported through the treatment of the underlying diseases or comorbidities. CASE PRESENTATION: We present a case of a 45-year-old woman with a chief complaint of dyspnoea, eventually diagnosed with severe PAH in the setting of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome that was successfully treated with thalidomide and dexamethasone. DISCUSSION: Our case suggests that it would be important to consider associated syndromes when a diagnosis of PH is made, because treatment of the underlying condition may lead to improvement in PAH.
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spelling pubmed-61771002019-04-24 Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report Tanaka, Toshikazu D Misawa, Sonoko Yoshimura, Michihiro Kuwabara, Satoshi Eur Heart J Case Rep Case Reports INTRODUCTION: Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease characterized by the remodelling of distal pulmonary arteries in the absence of other cardiopulmonary disease, usually leading to right ventricular failure. Given the current European Society of Cardiology and the European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension (PH), most of the patients with severe PAH usually require to have a lifelong combination therapy that includes prostacyclin, phosphodiesterase-5 inhibitor, and endothelin receptor antagonist. However, the reversibility of PAH has been reported through the treatment of the underlying diseases or comorbidities. CASE PRESENTATION: We present a case of a 45-year-old woman with a chief complaint of dyspnoea, eventually diagnosed with severe PAH in the setting of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome that was successfully treated with thalidomide and dexamethasone. DISCUSSION: Our case suggests that it would be important to consider associated syndromes when a diagnosis of PH is made, because treatment of the underlying condition may lead to improvement in PAH. Oxford University Press 2018-04-27 /pmc/articles/PMC6177100/ /pubmed/31020130 http://dx.doi.org/10.1093/ehjcr/yty051 Text en © The Author(s) 2018. Published by Oxford University Press on behalf of the European Society of Cardiology. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Reports
Tanaka, Toshikazu D
Misawa, Sonoko
Yoshimura, Michihiro
Kuwabara, Satoshi
Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report
title Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report
title_full Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report
title_fullStr Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report
title_full_unstemmed Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report
title_short Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report
title_sort reversal of pulmonary arterial hypertension in poems syndrome with thalidomide: a case report
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6177100/
https://www.ncbi.nlm.nih.gov/pubmed/31020130
http://dx.doi.org/10.1093/ehjcr/yty051
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