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Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients
OBJECTIVE: A new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes between idiopathic-relapsing polychondritis (I-RP) and VEXAS-r...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BMJ Publishing Group
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9315905/ https://www.ncbi.nlm.nih.gov/pubmed/35868738 http://dx.doi.org/10.1136/rmdopen-2022-002255 |
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author | Khitri, Mohamed-Yacine Guedon, Alexis F Georgin-Lavialle, Sophie Terrier, Benjamin Saadoun, David Seguier, Julie le Besnerais, Maelle De Moreuil, Claire Denis, Guillaume Gerfaud-Valentin, Mathieu Allain, Jean Sebastien Maria, Alexandre Bouillet, Laurence Grobost, Vincent Galland, Joris Kosmider, Olivier Dumont, Anael Devaux, Mathilde Subran, Benjamin Schmidt, Jean Marianetti-Guingel, Paola Audia, Sylvain Palat, Sylvain Roux-Sauvat, Marielle Jachiet, Vincent Hirsch, Pierre Fain, Olivier Mekinian, Arsène |
author_facet | Khitri, Mohamed-Yacine Guedon, Alexis F Georgin-Lavialle, Sophie Terrier, Benjamin Saadoun, David Seguier, Julie le Besnerais, Maelle De Moreuil, Claire Denis, Guillaume Gerfaud-Valentin, Mathieu Allain, Jean Sebastien Maria, Alexandre Bouillet, Laurence Grobost, Vincent Galland, Joris Kosmider, Olivier Dumont, Anael Devaux, Mathilde Subran, Benjamin Schmidt, Jean Marianetti-Guingel, Paola Audia, Sylvain Palat, Sylvain Roux-Sauvat, Marielle Jachiet, Vincent Hirsch, Pierre Fain, Olivier Mekinian, Arsène |
author_sort | Khitri, Mohamed-Yacine |
collection | PubMed |
description | OBJECTIVE: A new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes between idiopathic-relapsing polychondritis (I-RP) and VEXAS-relapsing polychondritis (VEXAS-RP). METHODS: Patients from French retrospective multicentre cohort of RP were separated into two groups: a VEXAS-RP and an I-RP. RESULTS: Compared with patients with I-RP (n=40), patients with VEXAS-RP (n=55) were men (96% vs 30%, p<0.001) and were older at diagnosis (66 vs 44 years, p<0.001). They had a greater prevalence of fever (60% vs 10%, p<0.001), of skin lesions (82% vs 20%, p<0.001), of ocular involvement (57% vs 28%, p=0.01), of pulmonary infiltrates (46% vs 0%, p<0.001), of heart involvement (11% vs 0%, p=0.0336) and with higher median C-reactive protein levels (64 mg/L vs 10 mg/L, p<0.001). Seventy-five per cent of the patients with VEXAS-RP had myelodysplastic syndrome (MDS) versus none in I-RP group. The glucocorticoids use, and the number of steroid sparing agents were similar in both groups, but patients with VEXAS-RP had more frequent refractory disease (remission obtained in 27% vs 90%, p<0001). VEXAS-RP was associated with higher risk of death: six patients (11%) died in the VEXAS-RP group after a median follow-up of 37 months and none in the I-RP group after a median follow-up of 92 months (p<0.05). CONCLUSION: We report the largest cohort of VEXAS-RP, characterised by high prevalence of male sex, fever, skin lesion, ocular involvement, pulmonary infiltration, heart involvement, older age and MDS association. |
format | Online Article Text |
id | pubmed-9315905 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BMJ Publishing Group |
record_format | MEDLINE/PubMed |
spelling | pubmed-93159052022-08-16 Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients Khitri, Mohamed-Yacine Guedon, Alexis F Georgin-Lavialle, Sophie Terrier, Benjamin Saadoun, David Seguier, Julie le Besnerais, Maelle De Moreuil, Claire Denis, Guillaume Gerfaud-Valentin, Mathieu Allain, Jean Sebastien Maria, Alexandre Bouillet, Laurence Grobost, Vincent Galland, Joris Kosmider, Olivier Dumont, Anael Devaux, Mathilde Subran, Benjamin Schmidt, Jean Marianetti-Guingel, Paola Audia, Sylvain Palat, Sylvain Roux-Sauvat, Marielle Jachiet, Vincent Hirsch, Pierre Fain, Olivier Mekinian, Arsène RMD Open Autoinflammatory Disorders OBJECTIVE: A new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes between idiopathic-relapsing polychondritis (I-RP) and VEXAS-relapsing polychondritis (VEXAS-RP). METHODS: Patients from French retrospective multicentre cohort of RP were separated into two groups: a VEXAS-RP and an I-RP. RESULTS: Compared with patients with I-RP (n=40), patients with VEXAS-RP (n=55) were men (96% vs 30%, p<0.001) and were older at diagnosis (66 vs 44 years, p<0.001). They had a greater prevalence of fever (60% vs 10%, p<0.001), of skin lesions (82% vs 20%, p<0.001), of ocular involvement (57% vs 28%, p=0.01), of pulmonary infiltrates (46% vs 0%, p<0.001), of heart involvement (11% vs 0%, p=0.0336) and with higher median C-reactive protein levels (64 mg/L vs 10 mg/L, p<0.001). Seventy-five per cent of the patients with VEXAS-RP had myelodysplastic syndrome (MDS) versus none in I-RP group. The glucocorticoids use, and the number of steroid sparing agents were similar in both groups, but patients with VEXAS-RP had more frequent refractory disease (remission obtained in 27% vs 90%, p<0001). VEXAS-RP was associated with higher risk of death: six patients (11%) died in the VEXAS-RP group after a median follow-up of 37 months and none in the I-RP group after a median follow-up of 92 months (p<0.05). CONCLUSION: We report the largest cohort of VEXAS-RP, characterised by high prevalence of male sex, fever, skin lesion, ocular involvement, pulmonary infiltration, heart involvement, older age and MDS association. BMJ Publishing Group 2022-07-22 /pmc/articles/PMC9315905/ /pubmed/35868738 http://dx.doi.org/10.1136/rmdopen-2022-002255 Text en © Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) . |
spellingShingle | Autoinflammatory Disorders Khitri, Mohamed-Yacine Guedon, Alexis F Georgin-Lavialle, Sophie Terrier, Benjamin Saadoun, David Seguier, Julie le Besnerais, Maelle De Moreuil, Claire Denis, Guillaume Gerfaud-Valentin, Mathieu Allain, Jean Sebastien Maria, Alexandre Bouillet, Laurence Grobost, Vincent Galland, Joris Kosmider, Olivier Dumont, Anael Devaux, Mathilde Subran, Benjamin Schmidt, Jean Marianetti-Guingel, Paola Audia, Sylvain Palat, Sylvain Roux-Sauvat, Marielle Jachiet, Vincent Hirsch, Pierre Fain, Olivier Mekinian, Arsène Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients |
title | Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients |
title_full | Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients |
title_fullStr | Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients |
title_full_unstemmed | Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients |
title_short | Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients |
title_sort | comparison between idiopathic and vexas-relapsing polychondritis: analysis of a french case series of 95 patients |
topic | Autoinflammatory Disorders |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9315905/ https://www.ncbi.nlm.nih.gov/pubmed/35868738 http://dx.doi.org/10.1136/rmdopen-2022-002255 |
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