Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies

Introduction Early diagnosis of systemic sclerosis (SSc) is important to start therapeutic interventions timely. Important risk factors for progression to SSc are the SSc-specific autoantibodies, of whom anti-centromere antibodies (ACA) and anti-topoisomerase I antibodies (ATA) are the most frequent...

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Main Authors: Cornelia F Allaart, Tom W J Huizinga, René E M Toes, Hans Ulrich Scherer, Jeska K de Vries-Bouwstra, Sophie I E Liem, Sam Neppelenbroek, Cynthia M Fehres, Brigitte A Wevers
Format: Article
Language:English
Published: BMJ Publishing Group 2023-02-01
Series:RMD Open
Online Access:https://rmdopen.bmj.com/content/9/1/e002827.full
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author Cornelia F Allaart
Tom W J Huizinga
René E M Toes
Hans Ulrich Scherer
Jeska K de Vries-Bouwstra
Sophie I E Liem
Sam Neppelenbroek
Cynthia M Fehres
Brigitte A Wevers
author_facet Cornelia F Allaart
Tom W J Huizinga
René E M Toes
Hans Ulrich Scherer
Jeska K de Vries-Bouwstra
Sophie I E Liem
Sam Neppelenbroek
Cynthia M Fehres
Brigitte A Wevers
author_sort Cornelia F Allaart
collection DOAJ
description Introduction Early diagnosis of systemic sclerosis (SSc) is important to start therapeutic interventions timely. Important risk factors for progression to SSc are the SSc-specific autoantibodies, of whom anti-centromere antibodies (ACA) and anti-topoisomerase I antibodies (ATA) are the most frequent. ATA is associated with a severe disease course. A more detailed characterisation of the ATA-response in SSc might increase insights in preclinical disease stages and improve prognostication. To address this we identified all patients with suspected very early ATA-positive SSc, defined as all patients who are ATA-positive not fulfilling American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 criteria, in the Leiden Combined Care in Systemic Sclerosis (CCISS)-cohort and found very low numbers.Methods This triggered us to search the literature on the ATA prevalence in patients with suspected very early SSc and contribution of the SSc-specific autoantibodies to progression from suspected very early to definite SSc. To increase insights on the ATA-response in suspected very early SSc, we then evaluated the association between the ATA-response and time between onset of Raynaud’s phenomenon (RP) and first non-RP symptom, as a proxy for progressing to definite SSc, in all patients with ATA-positive SSc from the Leiden CCISS-cohort.Results In short, included studies show that prevalence of ATA is much lower in suspected very early SSc than in populations fulfilling ACR/EULAR 2013 criteria. After 1–15 years of follow-up, only 52% of the patients with suspected very early SSc progress to definite SSc. ATA-IgG levels tend to be higher in patients with ATA-positive SSc with more rapid disease progression.Conclusion Although a role of ATA in disease progression is suggested, more studies on the ATA response in suspected very early SSc are warranted.
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spelling doaj.art-b60d35cbf22c4b359a2ebf2422ce84b22023-02-17T20:30:09ZengBMJ Publishing GroupRMD Open2056-59332023-02-019110.1136/rmdopen-2022-002827Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodiesCornelia F Allaart0Tom W J Huizinga1René E M Toes2Hans Ulrich Scherer3Jeska K de Vries-Bouwstra4Sophie I E Liem5Sam Neppelenbroek6Cynthia M Fehres7Brigitte A Wevers8Department of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Clinical Chemistry and Laboratory Medicine, Leiden University Medical Center, Leiden, The NetherlandsIntroduction Early diagnosis of systemic sclerosis (SSc) is important to start therapeutic interventions timely. Important risk factors for progression to SSc are the SSc-specific autoantibodies, of whom anti-centromere antibodies (ACA) and anti-topoisomerase I antibodies (ATA) are the most frequent. ATA is associated with a severe disease course. A more detailed characterisation of the ATA-response in SSc might increase insights in preclinical disease stages and improve prognostication. To address this we identified all patients with suspected very early ATA-positive SSc, defined as all patients who are ATA-positive not fulfilling American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 criteria, in the Leiden Combined Care in Systemic Sclerosis (CCISS)-cohort and found very low numbers.Methods This triggered us to search the literature on the ATA prevalence in patients with suspected very early SSc and contribution of the SSc-specific autoantibodies to progression from suspected very early to definite SSc. To increase insights on the ATA-response in suspected very early SSc, we then evaluated the association between the ATA-response and time between onset of Raynaud’s phenomenon (RP) and first non-RP symptom, as a proxy for progressing to definite SSc, in all patients with ATA-positive SSc from the Leiden CCISS-cohort.Results In short, included studies show that prevalence of ATA is much lower in suspected very early SSc than in populations fulfilling ACR/EULAR 2013 criteria. After 1–15 years of follow-up, only 52% of the patients with suspected very early SSc progress to definite SSc. ATA-IgG levels tend to be higher in patients with ATA-positive SSc with more rapid disease progression.Conclusion Although a role of ATA in disease progression is suggested, more studies on the ATA response in suspected very early SSc are warranted.https://rmdopen.bmj.com/content/9/1/e002827.full
spellingShingle Cornelia F Allaart
Tom W J Huizinga
René E M Toes
Hans Ulrich Scherer
Jeska K de Vries-Bouwstra
Sophie I E Liem
Sam Neppelenbroek
Cynthia M Fehres
Brigitte A Wevers
Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies
RMD Open
title Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies
title_full Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies
title_fullStr Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies
title_full_unstemmed Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies
title_short Progression from suspected to definite systemic sclerosis and the role of anti-topoisomerase I antibodies
title_sort progression from suspected to definite systemic sclerosis and the role of anti topoisomerase i antibodies
url https://rmdopen.bmj.com/content/9/1/e002827.full
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