Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients

Introduction Systemic sclerosis (SSc) is a rare autoimmune disease currently classified into two subgroups based on skin extension. The aim of this study was to determine in a large cohort whether the determination of autoantibody (AAb) profile among a full antinuclear AAbs panel including nine spec...

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Main Authors: Thierry Martin, David Launay, Thomas Barnetche, Christian Agard, Vincent Sobanski, Marie-Elise Truchetet, Jean-Loup Pennaforte, Philippe Guilpain, Arnaud Hot, Coralie Barbe, Alexandre Maria, Amélie Servettaz, Cécile Contin-Bordes, Manuelle Viguier, Kévin Didier, Ailsa Robbins, Romain Fort, Damien Jolly, Delphine Giusti
Format: Article
Language:English
Published: BMJ Publishing Group 2024-11-01
Series:RMD Open
Online Access:https://rmdopen.bmj.com/content/10/4/e004580.full
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author Thierry Martin
David Launay
Thomas Barnetche
Christian Agard
Vincent Sobanski
Marie-Elise Truchetet
Jean-Loup Pennaforte
Philippe Guilpain
Arnaud Hot
Coralie Barbe
Alexandre Maria
Amélie Servettaz
Cécile Contin-Bordes
Manuelle Viguier
Kévin Didier
Ailsa Robbins
Romain Fort
Damien Jolly
Delphine Giusti
author_facet Thierry Martin
David Launay
Thomas Barnetche
Christian Agard
Vincent Sobanski
Marie-Elise Truchetet
Jean-Loup Pennaforte
Philippe Guilpain
Arnaud Hot
Coralie Barbe
Alexandre Maria
Amélie Servettaz
Cécile Contin-Bordes
Manuelle Viguier
Kévin Didier
Ailsa Robbins
Romain Fort
Damien Jolly
Delphine Giusti
author_sort Thierry Martin
collection DOAJ
description Introduction Systemic sclerosis (SSc) is a rare autoimmune disease currently classified into two subgroups based on skin extension. The aim of this study was to determine in a large cohort whether the determination of autoantibody (AAb) profile among a full antinuclear AAbs panel including nine specificities had a higher impact than skin phenotype on stratifying the risk of organ involvement and mortality in SSc.Methods Data for patients with SSc followed in seven French university hospitals were retrospectively analysed in terms of skin phenotype, AAbs (anti-topoisomerase I (ATA), anticentromere (ACA), anti-RNA polymerase III (anti-RNAPIII), anti-U1RNP, anti-U3RNP, anti-Pm/Scl, anti-Ku, anti-Th/To, anti-NOR90), organ involvement and mortality. Multivariate analyses were performed to identify independent factors associated with organ involvement and mortality.Results We included 1605 patients with SSc (367 with diffuse cutaneous SSc). On multivariate analysis, ATAs were associated with interstitial lung disease and mortality (OR=3.27 (95% CI 2.42 to 4.42); HR=1.9 (95% CI 1.01 to 3.58)), anti-RNAPIII with scleroderma renal crisis and mortality (OR=7.05 (95% CI 2.98 to 16.72); HR=2.35 (95% CI 1.12 to 4.93)), anti-U1RNP with arthritis (OR=3.79 (95% CI 2.16 to 6.67)), anti-Pm/Scl and anti-Ku with myositis (OR=7.09 (95% CI 3.87 to 12.98) and 7.99 (95% CI 2.41 to 26.46)). The skin phenotype was not associated with survival or organ involvement on multivariate analysis without stepwise selection.Conclusion This study unravels, by contrast with skin phenotype, a strong association between AAbs specificities, organ involvement and outcome in SSc and suggests that patients’ classification based on only skin extension is not sufficient for defining prognosis and phenotype.
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spelling doaj-art-09c75251d03e4764bb12ee52e1a45dd82025-08-20T02:03:04ZengBMJ Publishing GroupRMD Open2056-59332024-11-0110410.1136/rmdopen-2024-004580Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patientsThierry Martin0David Launay1Thomas Barnetche2Christian Agard3Vincent Sobanski4Marie-Elise Truchetet5Jean-Loup Pennaforte6Philippe Guilpain7Arnaud Hot8Coralie Barbe9Alexandre Maria10Amélie Servettaz11Cécile Contin-Bordes12Manuelle Viguier13Kévin Didier14Ailsa Robbins15Romain Fort16Damien Jolly17Delphine Giusti18Department of Clinical Immunology and Internal Medicine, Strasbourg University Hospitals, Strasbourg, Grand Est, FranceUniv. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, FranceRheumatology Department, FHU ACRONIM, University Hospital Centre Bordeaux, Bordeaux, Nouvelle-Aquitaine, FranceService de Médecine Interne, Centre Hospitalier Universitaire de Nantes, Nantes, FranceINFINITE—Institute for Translational Research in Inflammation, Lille, FranceRheumatology Department, FHU ACRONIM, University Hospital Centre Bordeaux, Bordeaux, Nouvelle-Aquitaine, FranceDepartment of Internal Medicine, University Hospital Centre Reims, Reims, Grand Est, FranceDepartment of Internal Medicine-Multiorganic Diseases, Local Referral Center for Auto-immune Diseases, Saint-Eloi Hospital, University Hospital Centre of Montpellier, Montpellier, FranceDepartment of Internal Medicine, CHU Edouard Herriot, Hospices Civils de Lyon, University Hospital Centre Lyon, Lyon, Auvergne-Rhône-Alpes, FranceHealth Research University Department, Université de Reims Champagne-Ardenne, Reims, FranceDepartment of Internal Medicine - Multi-organ Diseases, St Eloi Hospital, Montpellier University Hospital, Univ Montpellier, Montpellier, FranceDepartment of Internal Medicine, Infectious Diseases, and Clinical Immunology, University Hospital Centre Reims, Reims, Grand Est, FranceCIRID, UMR CNRS-5164, ImmunoConcEpT, Bordeaux University, Bordeaux, FranceDepartment of Dermatology, University Hospital Centre Reims, Reims, Grand Est, FranceDepartment of Internal Medicine, Infectious Diseases, and Clinical Immunology, University Hospital Centre Reims, Reims, Grand Est, FranceDepartment of Internal Medicine, Infectious Diseases, and Clinical Immunology, University Hospital Centre Reims, Reims, Grand Est, FranceDepartment of Internal Medicine, CHU Edouard Herriot, Hospices Civils de Lyon, University Hospital Centre Lyon, Lyon, Auvergne-Rhône-Alpes, FranceClinical Epidemiology Department, University Hospital Centre Reims, Reims, Grand Est, FranceUR7509 - IRMAIC, University of Reims Champagne-Ardenne, Reims, Grand Est, FranceIntroduction Systemic sclerosis (SSc) is a rare autoimmune disease currently classified into two subgroups based on skin extension. The aim of this study was to determine in a large cohort whether the determination of autoantibody (AAb) profile among a full antinuclear AAbs panel including nine specificities had a higher impact than skin phenotype on stratifying the risk of organ involvement and mortality in SSc.Methods Data for patients with SSc followed in seven French university hospitals were retrospectively analysed in terms of skin phenotype, AAbs (anti-topoisomerase I (ATA), anticentromere (ACA), anti-RNA polymerase III (anti-RNAPIII), anti-U1RNP, anti-U3RNP, anti-Pm/Scl, anti-Ku, anti-Th/To, anti-NOR90), organ involvement and mortality. Multivariate analyses were performed to identify independent factors associated with organ involvement and mortality.Results We included 1605 patients with SSc (367 with diffuse cutaneous SSc). On multivariate analysis, ATAs were associated with interstitial lung disease and mortality (OR=3.27 (95% CI 2.42 to 4.42); HR=1.9 (95% CI 1.01 to 3.58)), anti-RNAPIII with scleroderma renal crisis and mortality (OR=7.05 (95% CI 2.98 to 16.72); HR=2.35 (95% CI 1.12 to 4.93)), anti-U1RNP with arthritis (OR=3.79 (95% CI 2.16 to 6.67)), anti-Pm/Scl and anti-Ku with myositis (OR=7.09 (95% CI 3.87 to 12.98) and 7.99 (95% CI 2.41 to 26.46)). The skin phenotype was not associated with survival or organ involvement on multivariate analysis without stepwise selection.Conclusion This study unravels, by contrast with skin phenotype, a strong association between AAbs specificities, organ involvement and outcome in SSc and suggests that patients’ classification based on only skin extension is not sufficient for defining prognosis and phenotype.https://rmdopen.bmj.com/content/10/4/e004580.full
spellingShingle Thierry Martin
David Launay
Thomas Barnetche
Christian Agard
Vincent Sobanski
Marie-Elise Truchetet
Jean-Loup Pennaforte
Philippe Guilpain
Arnaud Hot
Coralie Barbe
Alexandre Maria
Amélie Servettaz
Cécile Contin-Bordes
Manuelle Viguier
Kévin Didier
Ailsa Robbins
Romain Fort
Damien Jolly
Delphine Giusti
Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients
RMD Open
title Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients
title_full Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients
title_fullStr Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients
title_full_unstemmed Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients
title_short Impact of autoantibody status on stratifying the risk of organ involvement and mortality in SSc: experience from a multicentre French cohort of 1605 patients
title_sort impact of autoantibody status on stratifying the risk of organ involvement and mortality in ssc experience from a multicentre french cohort of 1605 patients
url https://rmdopen.bmj.com/content/10/4/e004580.full
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