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AB0614 A Comphehensive Analysis of 12 Disease-Specific Autoantibodies in Greek Patients with Systemic Sclerosis
  1. C. Liaskos1,
  2. E. Marou1,
  3. T. Simopoulou1,
  4. M. Barmakoudi1,
  5. T. Scheper2,
  6. W. Meyer2,
  7. C.G. Katsiari1,
  8. D. Bogdanos1,
  9. L.I. Sakkas1
  1. 1Rheumatology, Medical School, University of thessaly, Larissa, Greece
  2. 2Institute of Immunology, Euroimmun, Luebeck, Germany

Abstract

Background Patients with systemic sclerosis (SSc) are characterized by ANAs against various autoantigens, none of which is 100% sensitive for the disease. The need for profiling testing based on a combination of the most important SSc-related autoantigens has led to the development of commercial SSc-profile assays.

Objectives To determine the diagnostic and clinical relevance of systemic sclerosis (SSc)-related autoantibodies tested by a line SSc-profile assay in a well defined cohort of Caucasian SSc patients, all from Central Greece.

Methods 98 consecutive patients with SSc (80 female, 18 male, mean age 57,9±14,9 y) including 60 patients with diffuse cutaneous SSc (dcSSc) and 38 with limited cutaneous SSc (lcSSc) were analyzed by a profile SSc line assay (Euroimmun) for autoantibodies against 12 autoantigens: Scl-70, Centromere (A, B), RNA polymerase III (subunits 11 & 155), fibrillarin, NOR90, Th/To, PM/Scl 100, PM/Scl75, Ku and PDGFR.

Results ANA by indirect immunofluorescence was present in 98 (98%) patients with SSc. Overall, 81 (82.6%) patients had abs against at least one of the 12 autoantigens. Anti-Scl-70 abs were present in 49 (49.4%) SSc patients, anti-centromere A in 22 (22.5%), anti-centromere B in 22 (22.5%, all anti-centromere A positive) anti-RNA polymerase III RP155) in 18 (18.3%) anti-RNA polymerase III RP11 in 14 (14.3%), anti-fibrillarin in 3 (3.1%), anti-Ku in 5 (5.1%), anti-NOR90 in 7 (7.1%), anti-PM/Scl100 in 4 (4.1%), anti-PM/Scl75 in 7 (7.1%), anti-Th/To in 4 (4.1%) and anti-PGDFR in 1 (1%) SSc patients. Overall, the most frequent anti-Scl-70, anti-centromere or anti-RNA polymerase III abs were present in 81 (81%) SSc patients while 6 (6.1%) lacked those antibodies and were positive for at least one of the other 9. Anti-Scl-70 were associated with diffuse SSc and ACA with limited SSc (p<0.001 for both). Positivity of Scl-70 was also correlated with the presence of lung fibrosis (p=0.004) and tended to be associated with the presence of digital ulcers (p=0.073). Anti-centromere abs were less prevalent in SSc patients with lung fibrosis (p=0.004). Anti-PM/Scl75 abs tended to be associated with the presence of digital ulcers (p=0.055). Anti-NOR90 abs were more frequent in male SSc patients (p=0.019).

Conclusions Anti-Scl70, anti-centromere and anti-RNA polymerase are by far the most dominant autoantibodies in SSc but other disease-related autoantibodies can also be present in SSc patients lacking those three autoantibodies. The presence of anti-Scl-70 abs identifies patients with lung fibrosis and along with anti-PM/Scl75 tend to be associated with the presence of digital ulcers.

Disclosure of Interest C. Liaskos: None declared, E. Marou: None declared, T. Simopoulou: None declared, M. Barmakoudi: None declared, T. Scheper Employee of: Euroimmun AG, W. Meyer Employee of: Euroimmun AG, C. Katsiari: None declared, D. Bogdanos: None declared, L. Sakkas: None declared

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