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Prevalence and clinical associations of anti-KU antibodies in patients with systemic sclerosis: a European EUSTAR - initiated multicentre case-control study
  1. B Rozman (kc.lj.rozman{at}siol.net)
  1. Dr Peter Drzaj Hospital, Slovenia
    1. S Èuènik (sasa.cucnik{at}guest.arnes.si)
    1. Univ.Med.Centre, Slovenia
      1. S Sodin-Semrl (ssodin1{at}yahoo.com)
      1. Univ.Med.Centre, Slovenia
        1. L Czirják (laszlo.czirjak{at}aok.pte.hu)
        1. University of Pecs, Hungary
          1. C Varjú
          1. University of Pecs, Hungary
            1. O Distler (oliver.distler{at}usz.ch)
            1. Center of Experimental Rheumatology, Switzerland
              1. D Huscher (huscher{at}drfz.de)
              1. German Rheumatism Research Centre, Berlin, Germany, Germany
                1. M Aringer (martin.aringer{at}meduniwien.ac.at)
                1. Internal Medicine III, Medical University of Vienna, Austria
                  1. G Steiner (guenter.steiner{at}meduniwien.ac.at)
                  1. University of Vienna, Austria
                    1. M Matucci-Cerinic (cerinic{at}unifi.it)
                    1. University of Florence, Italy
                      1. S Guiducci (laboratorio.matucci{at}unifi.it)
                      1. University of Florence, Italy
                        1. B Stamenkoviæ (ackana{at}junis.ni.ac.yu)
                        1. Institute for prevention and treatment rheumatic and cardiovascular dis, Serbia and Montenegro
                          1. A Stankoviæ (ackana{at}junis.ni.ac.yu)
                          1. Institute for prevention and treatment rheumatic and cardiovascular dis, Serbia and Montenegro
                            1. T Kveder (tanja.kveder{at}kclj.si)
                            1. University Medical Centre, Slovenia

                              Abstract

                              Objectives: To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatologic centres and to evaluate their clinical and serologic characteristics.

                              Methods: Sera of 625 consecutive patients with either limited cutaneous or diffuse cutaneous SSc were tested for antibodies to Ku antigen together with other extractable nuclear antigens by counter-immunoelectrophoresis. A case-control design with calculation of Bootstrap 95% confidence intervals derived from anti-Ku negative control patients was used to evaluate clinical associations of anti-Ku antibodies. Sera of anti-Ku positive SSc patients and control group were additionally tested by immunofluorescence on Hep-2 cell substrates and line immunoassay.

                              Results: Anti-Ku antibodies were found in sera of 14/625 (2.2%) SSc patients. Ten out of fourteen anti-Ku positive SSc patients had no other anti-ENA antibodies detected by counterimmuno-electrophoresis. Using the case control design, anti-Ku antibodies were significantly associated with musculoskeletal manifestations such as clinical markers of myositis, arthritis, and joint contractures. In addition, a significant negative correlation of anti-Ku antibodies was found with vascular manifestation such as fingertip ulcers and teleangiectasias. There was a striking absence of anti-centromere antibodies as well as anti-PM/Scl antibodies in anti-Ku positive patients. As expected, anti-Scl-70 and punctate nucleolar immunofluorescence pattern were present only in single cases.

                              Conclusion: This is the largest cohort to date focusing on the prevalence of anti-Ku antibodies in SSc patients. The case control approach was able to demonstrate a clinically distinct subset of anti-Ku positive SSc patients with only relative clinical differences in skeletal features. However, the notable exceptions were signs of myositis. This shows the importance of anti-Ku antibody detection for the prediction of this specific clinical subset.

                              • anti-Ku antibodies
                              • case control approach
                              • multicentre study
                              • systemic sclerosis

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