Background Autoantibodies (autoAbs) help in diagnosis and predicting clinical phenotypes in systemic sclerosis (SSc).
Objectives To determine the clinical utility of 13 SSc-related autoAbs in SSc patients in Greece.
Methods 131 consecutive patients with SSc (111 female, mean age 58.1±14 years; 49 with diffused cutaneous SSc [dcSSc] and 82 with limited cutaneous SSc [lcSSc]) were analyzed by a multiplex line immunoassay (Euroimmun) for autoAbs against 13 SSc-related antigens. Twenty two patients with primary Raynaud phenomenon (RP), and 22 healthy controls were also analyzed
Results ANA by indirect immunofluorescence was present in 128 (97.7%) patients with SSc. Excluding anti-Ro52, 113 (89.3%) SSc patients were positive for at least one autoAb: anti-Topo I abs in 54 (41.2%), anti-CENPA in 37 (28.2%, all reactive with CENPB), anti-RNA polymerase III (RP11) in 19 (14.5%), anti-RNA polymerase III (RP155) in 13 (9.9%), anti-fibrillarin in 4 (3.1%), anti-Ku in 6 (4.6%), anti-NOR90 in 8 (6.1%), anti-PM/Scl100 in 2 (1.5%), and anti-PM/Scl75 in 4 (3.1%). There was no immunoreactivity for Th/To or PDGFR. Overall, 102 (77.9%) SSc patients had autoAbs against Topo I, CENPA or CENPB, RP11 or RP155. Anti-Topo I abs were strongly associated with dcSSc, interstitial lung disease (ILD) (p<0.001), and pulmonary arterial hypertension (PAH) (p=0.019). Anti-CENB abs were associated with lcSSc, and negatively associated with ILD. Anti-RP11 and anti-NOR90 abs were associated with male gender, and anti-NOR90 associated with ILD. In PR individuals and healthy individuals anti-Ro52 immunoreactivity was found in 9.1% and 13.6%, respectively, but no other immunoreactivity was detected.
Conclusions Anti-Scl70, anti-CENP and anti-RNA pol III are the most prevalent autoAbs in SSc. Anti-Topo I and anti-NOR90 abs are associated with ILD and/or PAH.
Disclosure of Interest C. Liaskos: None declared, E. Marou: None declared, T. Simopoulou: None declared, M. Barmakoudi: None declared, G. Efthymiou: None declared, T. Scheper Employee of: Euroimmun AG, W. Meyer Employee of: Euroimmun AG, D. Bogdanos: None declared, L. Sakkas: None declared