Disease-related autoantibody profile in patients with systemic sclerosis

Abstract

Background: Autoantibodies (autoAbs) help in diagnosis and predicting clinical phenotypes in systemic sclerosis (SSc).

Aim of the study: To determine the clinical utility of 13 SSc-related autoAbs in SSc patients.

Material and methods: A total of 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 analysed by a multiplex line immunoassay (Euroimmun) for autoantibodies (autoAbs) against 13 SSc-related antigens. A total of 22 patients with primary Raynaud phenomenon (RP), and 22 healthy controls were also analysed.

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-Topoisomerase I (anti-Topo) I abs in 54 (41.2%), anti-centromere proteins (anti-CENP) in 37 (28.2%, all reactive with centromere protein-A (CENPA) and centromere protein B (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-nucleolus-organizing region (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 platelet-derived growth factor receptor (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 < .001), pulmonary hypertension (PH) (p = .019) and ILD-PH (p = .003). Anti-CENPB 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.

Conclusions: Anti-Topo I, 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.

Keywords:

• Autoimmunity
• autoantibodies
• fibrosis
• line assay
• scleroderma
• ulcers

Acknowledgements

This work was supported in part by a grant from ELKE (Research Committee), University of Thessaly. We thank Euroimmun AG for providing free of charge reagents and kits.

Disclosure statement

T. Scheper and W. Meyer are employees of Euroimmun; all other authors do not have anything to disclose

Part of this work was communicated in 2016 EULAR's Annual European Congress of Rheumatology (accepted as Liaskos C, Marou E, Simopoulou T, Barmakoudi M, Scheper T, Meyer W, Katsiari CG, Bogdanos DP, Sakkas LI. Comphehensive Analysis of 12 Disease-Specific Autoantibodies in Greek Patients with Systemic Sclerosis. Ann Rheum Dis 2016 Vol 75 suppl 2 http://dx.doi.org/10.1136/annrheumdis-2016-eular.4355).

Additional information

Funding

This work was supported in part by a grant from ELKE (Research Committee), University of Thessaly. We thank Euroimmun AG for providing free of charge reagents and kits.