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Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature

Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. This study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hyperte...

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Autores principales: Sanges, Sébastien, Yelnik, Cécile M., Sitbon, Olivier, Benveniste, Olivier, Mariampillai, Kuberaka, Phillips-Houlbracq, Mathilde, Pison, Christophe, Deligny, Christophe, Inamo, Jocelyn, Cottin, Vincent, Mouthon, Luc, Launay, David, Lambert, Marc, Hatron, Pierre-Yves, Rottat, Laurence, Humbert, Marc, Hachulla, Eric
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5265921/
https://www.ncbi.nlm.nih.gov/pubmed/27684828
http://dx.doi.org/10.1097/MD.0000000000004911
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author Sanges, Sébastien
Yelnik, Cécile M.
Sitbon, Olivier
Benveniste, Olivier
Mariampillai, Kuberaka
Phillips-Houlbracq, Mathilde
Pison, Christophe
Deligny, Christophe
Inamo, Jocelyn
Cottin, Vincent
Mouthon, Luc
Launay, David
Lambert, Marc
Hatron, Pierre-Yves
Rottat, Laurence
Humbert, Marc
Hachulla, Eric
author_facet Sanges, Sébastien
Yelnik, Cécile M.
Sitbon, Olivier
Benveniste, Olivier
Mariampillai, Kuberaka
Phillips-Houlbracq, Mathilde
Pison, Christophe
Deligny, Christophe
Inamo, Jocelyn
Cottin, Vincent
Mouthon, Luc
Launay, David
Lambert, Marc
Hatron, Pierre-Yves
Rottat, Laurence
Humbert, Marc
Hachulla, Eric
author_sort Sanges, Sébastien
collection PubMed
description Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. This study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hypertension (PH) Registry, to identify IIM features associated with the presence of PAH, and to describe treatment modalities of these patients. All cases of IIM-PAH were retrieved from the French PH Registry, which gathers PH patients prospectively enrolled by 27 referral hospital centers across France. Patients were excluded if they had an extensive ILD or overlap syndrome. Characteristics of IIM-PAH patients were compared with a control group of IIM patients without PH. Among the 5223 PH patients in the Registry, 34 had a diagnosis of IIM. Among them, 3 IIM-PAH patients (2 females and 1 male) had no evidence of extensive ILD or overlap syndrome, and were included in this study. In these 3 patients, dermatomyositis (DM) was the only identified IIM. One patient had autoantibodies classically associated with IIM (anti-Ku). PAH had always developed after IIM onset, was severe in all cases, and led to a marked functional impairment. By pooling our cases with 6 patients previously reported in the literature, and comparing them with a control cohort of 35 IIM patients without PH, we identify several IIM characteristics possibly associated with PAH occurrence, including DM subtype (78% vs 46%; P = 0.02), skin involvement (P = 0.04), anti-SSA antibodies (P = 0.05), and peripheral microangiopathy (P = 0.06). Overall, IIM-PAH patients were managed by corticosteroids and/or immunosuppressants, either alone or combined with PAH therapy. Patients did not seem to respond to IIM treatment alone. Our study reports for the first time the rare but possible association of PAH and IIM in a large prospective PH Registry. In that setting, PAH seems associated with DM, skin involvement, peripheral microangiopathy, and anti-SSA positivity. The best therapeutic strategy for IIM-PAH remains to be defined.
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spelling pubmed-52659212017-02-06 Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature Sanges, Sébastien Yelnik, Cécile M. Sitbon, Olivier Benveniste, Olivier Mariampillai, Kuberaka Phillips-Houlbracq, Mathilde Pison, Christophe Deligny, Christophe Inamo, Jocelyn Cottin, Vincent Mouthon, Luc Launay, David Lambert, Marc Hatron, Pierre-Yves Rottat, Laurence Humbert, Marc Hachulla, Eric Medicine (Baltimore) 6900 Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. This study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hypertension (PH) Registry, to identify IIM features associated with the presence of PAH, and to describe treatment modalities of these patients. All cases of IIM-PAH were retrieved from the French PH Registry, which gathers PH patients prospectively enrolled by 27 referral hospital centers across France. Patients were excluded if they had an extensive ILD or overlap syndrome. Characteristics of IIM-PAH patients were compared with a control group of IIM patients without PH. Among the 5223 PH patients in the Registry, 34 had a diagnosis of IIM. Among them, 3 IIM-PAH patients (2 females and 1 male) had no evidence of extensive ILD or overlap syndrome, and were included in this study. In these 3 patients, dermatomyositis (DM) was the only identified IIM. One patient had autoantibodies classically associated with IIM (anti-Ku). PAH had always developed after IIM onset, was severe in all cases, and led to a marked functional impairment. By pooling our cases with 6 patients previously reported in the literature, and comparing them with a control cohort of 35 IIM patients without PH, we identify several IIM characteristics possibly associated with PAH occurrence, including DM subtype (78% vs 46%; P = 0.02), skin involvement (P = 0.04), anti-SSA antibodies (P = 0.05), and peripheral microangiopathy (P = 0.06). Overall, IIM-PAH patients were managed by corticosteroids and/or immunosuppressants, either alone or combined with PAH therapy. Patients did not seem to respond to IIM treatment alone. Our study reports for the first time the rare but possible association of PAH and IIM in a large prospective PH Registry. In that setting, PAH seems associated with DM, skin involvement, peripheral microangiopathy, and anti-SSA positivity. The best therapeutic strategy for IIM-PAH remains to be defined. Wolters Kluwer Health 2016-09-30 /pmc/articles/PMC5265921/ /pubmed/27684828 http://dx.doi.org/10.1097/MD.0000000000004911 Text en Copyright © 2016 the Author(s). Published by Wolters Kluwer Health, Inc. All rights reserved. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
spellingShingle 6900
Sanges, Sébastien
Yelnik, Cécile M.
Sitbon, Olivier
Benveniste, Olivier
Mariampillai, Kuberaka
Phillips-Houlbracq, Mathilde
Pison, Christophe
Deligny, Christophe
Inamo, Jocelyn
Cottin, Vincent
Mouthon, Luc
Launay, David
Lambert, Marc
Hatron, Pierre-Yves
Rottat, Laurence
Humbert, Marc
Hachulla, Eric
Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature
title Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature
title_full Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature
title_fullStr Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature
title_full_unstemmed Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature
title_short Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature
title_sort pulmonary arterial hypertension in idiopathic inflammatory myopathies: data from the french pulmonary hypertension registry and review of the literature
topic 6900
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5265921/
https://www.ncbi.nlm.nih.gov/pubmed/27684828
http://dx.doi.org/10.1097/MD.0000000000004911
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