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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Format: | Article |
Language: | English |
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BMJ Publishing Group
2023-02-01
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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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institution | Directory Open Access Journal |
issn | 2056-5933 |
language | English |
last_indexed | 2024-04-10T09:35:42Z |
publishDate | 2023-02-01 |
publisher | BMJ Publishing Group |
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series | RMD Open |
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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