Seminars in arthritis and rheumatism · 2026
Autoantibody associations in patients with early diffuse cutaneous systemic sclerosis: the prospective registry of early systemic sclerosis.
СтатьяНазвание и аннотация: Перевод в очереди
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Agarwal Aradhna, Hassan Dahlia, Huang Suiyuan, Castelino Flavia V, Assassi Shervin, Domsic Robyn T, Frech Tracy M, Gordon Jessica K, Hant Faye N, Hinchcliff Monique E, Shah Ami A, Shanmugam Victoria K, Steen Virginia D, Khanna Dinesh, Bernstein Elana J
Аннотация
OBJECTIVE: Anti-RNA-polymerase-III (ARA) and anti-topoisomerase-I (ATA) autoantibodies are associated with diffuse cutaneous SSc (dcSSc). ARA is associated with rapidly progressive skin thickening, scleroderma renal crisis (SRC), and gastric antral vascular ectasia, while ATA is associated with severe interstitial lung disease (ILD). However, these associations were derived from heterogenous SSc cohorts. As SSc is now often diagnosed earlier, we aimed to ascertain whether these relationships hold true in early dcSSc and to examine autoantibody associations with organ involvement in early dcSSc.
METHODS: The Prospective Registry of Early Systemic Sclerosis (PRESS) includes adults with dcSSc ≤2 years from the first non-Raynaud's phenomenon symptom who met 2013 ACR/EULAR classification criteria for SSc. Participants were enrolled at 12 U.S. academic centers and evaluated every 6 months. Those with dual-positivity were excluded. Differences across autoantibody groups were assessed using appropriate parametric and nonparametric tests.
RESULTS: Of 303 enrolled, 233 participants were included (108 ARA+, 68 ATA+, 57 double-negative). Mean age was 51 years; 68% were female. Mean disease duration was 1.2 years. ATA+ participants had lower baseline FVC% predicted (76.0%vs 86.0% ARA+, p = 0.0011) and higher prevalence of ILD (69.6%vs 44.3% ARA+, p = 0.0029). ARA+ participants had higher baseline mRSS (25.0 vs 17.9 ATA+, p < 0.0001) and higher prevalence of SRC (14.4%vs 0.0% ATA+, p = 0.0024). Only the double-negative group had improvement in FVC% predicted over time (+1.39% per year, p = 0.03).
CONCLUSIONS: Autoantibody-specific differences in organ involvement were evident early. The double-negative group uniquely showed improvement in lung function over time.
METHODS: The Prospective Registry of Early Systemic Sclerosis (PRESS) includes adults with dcSSc ≤2 years from the first non-Raynaud's phenomenon symptom who met 2013 ACR/EULAR classification criteria for SSc. Participants were enrolled at 12 U.S. academic centers and evaluated every 6 months. Those with dual-positivity were excluded. Differences across autoantibody groups were assessed using appropriate parametric and nonparametric tests.
RESULTS: Of 303 enrolled, 233 participants were included (108 ARA+, 68 ATA+, 57 double-negative). Mean age was 51 years; 68% were female. Mean disease duration was 1.2 years. ATA+ participants had lower baseline FVC% predicted (76.0%vs 86.0% ARA+, p = 0.0011) and higher prevalence of ILD (69.6%vs 44.3% ARA+, p = 0.0029). ARA+ participants had higher baseline mRSS (25.0 vs 17.9 ATA+, p < 0.0001) and higher prevalence of SRC (14.4%vs 0.0% ATA+, p = 0.0024). Only the double-negative group had improvement in FVC% predicted over time (+1.39% per year, p = 0.03).
CONCLUSIONS: Autoantibody-specific differences in organ involvement were evident early. The double-negative group uniquely showed improvement in lung function over time.