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

被引:5
作者
Liem, Sophie I. E. [1 ]
Neppelenbroek, Sam [1 ]
Fehres, Cynthia M. [1 ]
Wevers, Brigitte A. [2 ]
Toes, Rene E. M. [1 ]
Allaart, Cornelia F. [1 ]
Huizinga, Tom W. J. [1 ]
Scherer, Hans Ulrich [1 ]
De Vries-Bouwstra, Jeska K. [1 ]
机构
[1] Leiden Univ, Med Ctr, Dept Rheumatol, Leiden, Netherlands
[2] Leiden Univ, Med Ctr, Dept Clin Chem & Lab Med, Leiden, Netherlands
来源
RMD OPEN | 2023年 / 9卷 / 01期
关键词
Autoantibodies; Scleroderma; Systemic; Autoimmunity; EULAR SCLERODERMA TRIALS; RAYNAUDS-PHENOMENON; ORGAN INVOLVEMENT; AUTOANTIBODIES; MANIFESTATIONS; CHALLENGE; CRITERIA; SIGN;
D O I
10.1136/rmdopen-2022-002827
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
IntroductionEarly 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.MethodsThis 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.ResultsIn 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.ConclusionAlthough 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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页数:10
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