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French recommendations for the management of systemic sclerosis
Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limb...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8310704/ https://www.ncbi.nlm.nih.gov/pubmed/34304732 http://dx.doi.org/10.1186/s13023-021-01844-y |
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author | Hachulla, Eric Agard, Christian Allanore, Yannick Avouac, Jerome Bader-Meunier, Brigitte Belot, Alexandre Berezne, Alice Bouthors, Anne-Sophie Condette-Wojtasik, Geraldine Constans, Joël De Groote, Pascal Diot, Elisabeth Dumas, Florence Jego, Patrick Joly, Francisca Launay, David Le Guern, Veronique Le Quintrec, Janine-Sophie Lescaille, Geraldine Meune, Christophe Moulin, Bruno Nguyen, Christelle Omeish, Nadine Pene, Frederic Richard, Marie-Aleth Rochefort, Juliette Roren, Alexandra Sitbon, Olivier Sobanski, Vincent Truchetet, Marie-Elise Mouthon, Luc |
author_facet | Hachulla, Eric Agard, Christian Allanore, Yannick Avouac, Jerome Bader-Meunier, Brigitte Belot, Alexandre Berezne, Alice Bouthors, Anne-Sophie Condette-Wojtasik, Geraldine Constans, Joël De Groote, Pascal Diot, Elisabeth Dumas, Florence Jego, Patrick Joly, Francisca Launay, David Le Guern, Veronique Le Quintrec, Janine-Sophie Lescaille, Geraldine Meune, Christophe Moulin, Bruno Nguyen, Christelle Omeish, Nadine Pene, Frederic Richard, Marie-Aleth Rochefort, Juliette Roren, Alexandra Sitbon, Olivier Sobanski, Vincent Truchetet, Marie-Elise Mouthon, Luc |
author_sort | Hachulla, Eric |
collection | PubMed |
description | Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis. |
format | Online Article Text |
id | pubmed-8310704 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-83107042021-07-26 French recommendations for the management of systemic sclerosis Hachulla, Eric Agard, Christian Allanore, Yannick Avouac, Jerome Bader-Meunier, Brigitte Belot, Alexandre Berezne, Alice Bouthors, Anne-Sophie Condette-Wojtasik, Geraldine Constans, Joël De Groote, Pascal Diot, Elisabeth Dumas, Florence Jego, Patrick Joly, Francisca Launay, David Le Guern, Veronique Le Quintrec, Janine-Sophie Lescaille, Geraldine Meune, Christophe Moulin, Bruno Nguyen, Christelle Omeish, Nadine Pene, Frederic Richard, Marie-Aleth Rochefort, Juliette Roren, Alexandra Sitbon, Olivier Sobanski, Vincent Truchetet, Marie-Elise Mouthon, Luc Orphanet J Rare Dis Position Statement Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis. BioMed Central 2021-07-26 /pmc/articles/PMC8310704/ /pubmed/34304732 http://dx.doi.org/10.1186/s13023-021-01844-y Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Position Statement Hachulla, Eric Agard, Christian Allanore, Yannick Avouac, Jerome Bader-Meunier, Brigitte Belot, Alexandre Berezne, Alice Bouthors, Anne-Sophie Condette-Wojtasik, Geraldine Constans, Joël De Groote, Pascal Diot, Elisabeth Dumas, Florence Jego, Patrick Joly, Francisca Launay, David Le Guern, Veronique Le Quintrec, Janine-Sophie Lescaille, Geraldine Meune, Christophe Moulin, Bruno Nguyen, Christelle Omeish, Nadine Pene, Frederic Richard, Marie-Aleth Rochefort, Juliette Roren, Alexandra Sitbon, Olivier Sobanski, Vincent Truchetet, Marie-Elise Mouthon, Luc French recommendations for the management of systemic sclerosis |
title | French recommendations for the management of systemic sclerosis |
title_full | French recommendations for the management of systemic sclerosis |
title_fullStr | French recommendations for the management of systemic sclerosis |
title_full_unstemmed | French recommendations for the management of systemic sclerosis |
title_short | French recommendations for the management of systemic sclerosis |
title_sort | french recommendations for the management of systemic sclerosis |
topic | Position Statement |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8310704/ https://www.ncbi.nlm.nih.gov/pubmed/34304732 http://dx.doi.org/10.1186/s13023-021-01844-y |
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