Abstract
Background:Systemic sclerosis (SSc) is an autoimmune rheumatic disease characterized by a wide range of disease-specific and disease-related autoantibodies (autoAbs). Profile assays have been developed and are currently in use to meet the demand for better characterization of all autoAbs found in SSc patients.Aim:To assess the clinical relevance of SSc-related autoantibodies in 158 patients with SSc, all from Central Greece, taking advantage of a multiparametric SSc autoantibody line immunoassay.Material and methods:158 consecutive patients with SSc (137 females, mean age 53.2 ± 10 years; 63 patients with dcSSc and 95 with lcSSc) from central Greece were included in the study. Eighteen patients with morphea were also included. Serum samples were analyzed by a profile SSc nucleoli line assay (Euroimmun) to detect Abs against 13 autoantigens: Scl-70, Centromere (A, B), RNA polymerase III (subunits 11 & 155), fibrillarin, NOR90, Th/To, PM/Scl 100, PM/Scl75, Ku, PDGFR and Ro52. Antinuclear autoAbs (ANAs) were detected by indirect immunofluorescence.Results:ANAs were detected in 97.5% of SSc patients. Reactivities to specific autoantigens were as follows: Topo I, 40.5%; CENP, 32.9%; Ro52, 21.5%; RP11, 8.9%; RP155, 13.3%; NOR 90, 4.4%; Ku 3.8%; PM-Scl75, 3.2%; PM-Scl100, 1.3%; Th/To, 1.3%; Fibrillarin, 1.3%; PDGFR 0%; Ro52 21.5%. Twenty-one of SSc did not have any of the main autoAbs, namely anti-Topo I, anti-CENP, anti-RNA pol III Abs.Conclusions:Multiparametric autoAb test provides positive SSc-associated autoAb reactivities in SSc patients negative for the three main autoAbs and this may prove of significance in early disease diagnosis.
Highlights
Systemic sclerosis (SSc) is an autoimmune disease characterized by widespread fibrosis and microvasculopathy, the latter being exemplified by Raynaud’s phenomenon and digital ulcers.[1]
Antinuclear antibodies (ANA) are present in most patients with SSc, but certain antinuclear autoAbs targeting specific nuclear autoantigens are associated with distinct cutaneous involvement subsets, namely diffuse cutaneous SSc and limited cutaneous SSc, cardinal clinical features of the disease.[5,9,10,11,12,13]
AutoAbs detected with these assays - including, apart from the conventional anticentromere Abs - against centromere protein B (CENPB) and CENPA, and anti-topoisomerase I, other less frequent disease-specific autoAbs, such as those against RNA polymerase III (RNA pol III) RNP11 and RNP155 subunits, anti-fibrillarin Abs, anti-PM-Scl Abs, against 100 and 75 kDa subunits (PM/Scl[100] and PM/ Scl75), anti-Th/To Abs, anti-nucleolus-organizing region (NOR) 90 Abs, anti-Ku Abs, anti-platelet-derived growth factor receptor (PDGFR) and anti-Ro52 Abs.[14,15]
Summary
Systemic sclerosis (SSc) is an autoimmune disease characterized by widespread fibrosis and microvasculopathy, the latter being exemplified by Raynaud’s phenomenon and digital ulcers.[1]. Serum samples were analyzed by a profile SSc nucleoli line assay (Euroimmun) to detect Abs against 13 autoantigens: Scl-70, Centromere (A, B), RNA polymerase III (subunits 11 & 155), fibrillarin, NOR90, Th/To, PM/Scl 100, PM/Scl[75], Ku, PDGFR and Ro52. Conclusions: Multiparametric autoAb test provides positive SSc-associated autoAb reactivities in SSc patients negative for the three main autoAbs and this may prove of significance in early disease diagnosis
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