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THU0064 Clinical signification of anti-ku antibody
  1. F Limbach1,
  2. J Goetz2,
  3. RM Javier1,
  4. JL Kuntz1,
  5. J Sibilia1
  1. 1Rheumatology
  2. 2Immunopathology, CHU Hautepierre, Strasbourg, France

Abstract

Background The Ku autoantigen is a DNA binding factor consisting of 70 and 80 kDa protein which form a heterodimer involved in repair of ds DNA breaks. Since the original description of anti-Ku antibody in patients with polymyositis-scleroderma overlap syndrome, this antibody has been showed to be associated with several autoimmune diseases.

Objectives To describe clinical features of 8 patients with anti-KU antibody.

Patient 12345678
Age (years) 2840443066416774
ENA (ELISA) Ku/RoKuKu/RNP/JO1KuKu/proteinase3KuKuKu
Clinical features Digital vasculitisSicca syndrome, polyarthritisMyalgia, pulmonary fibrosis, sclerodactylia, raynaud’s phenomenRaynaud’s phenomenExtra-capillar glomerulonephritis, polyarthralgia, rectal carcinomaPolyarthritisBronchopneumonia, Pulmonary embolismHand’s oedema, prostatic cancer
Diagnosis LupusSjögren syndromeMyositis-sclerodermaRaynaudWegener granulomatosisRheumatoid arthritisChronic obstructive lung diseaseProstatic cancer
RS3PE ?
Abstract THU0064 Table 1

Conclusion

  • anti-Ku antibody is not specific of a connective tissu disease and can also be observed in patients without any autoimmune disease (n°7).

  • In addition to previous described association, anti-Ku antibody can also be present in Wegener granulomatosis and idiopathic Raynaud’s phenomen.

  • Two of our patients had an evaluative cancer (n°5 and 8) and one (n°7) presented a stomach neoplasm 3 years before the detection of anti-Ku antibody. This finding needs further investigations to assess if anti-Ku antibody can be associated with or predictive of a neoplasm.

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