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SAT0210 Hierarchical Cluster Study of Patients with Anti-KU Antibodies Identifies Two Phenotypical Subgroups with Distinct Prognosis Recognized by Anti-DNA Status
  1. L. Spielman1,
  2. M. Schaeffer2,
  3. J. Goetz2,
  4. T. Martin2,
  5. J. Sibilia2,
  6. A. Meyer2
  1. 1Hôpitaux Universitaires de Strasbourg
  2. 2Hôpitaux Universitaires, Strasbourg, France

Abstract

Background The presence of anti-Ku antibody is associated with a wide variety of clinical manifestations and various outcomes.

Objectives We attended to refine anti-Ku associated disease by identifying subgroups of Ku-positive patients with similar clinico-biological features and prognosis.

Methods We reviewed clinical, biological characteristics, diagnosis and management of 24 patients with anti-Ku antibody. A multidimensional analyze was performed to highlight homogeneous groups of patients. We search for features that most strongly match with each group.

Results Anti-Ku positive patients had joint (n=19), muscle (n=10), lung (n=9), skin (n=9), renal (n=9), hematological (n=8), thrombotic (n=6), serous (n=6) and gastrointestinal (n=4) involvements. Associated antibodies were anti-DNA (n=7), -SSA (n=6), -RNP (n=4), -SSB (n=1), -Sm (n=1), rheumatoid factor (n=9) and ACPA (n=4). Diagnosis were overlap myositis (n=9), lupus erythematosus (n=8), Sjögren's syndrome (n=7), rheumatoid arthritis (n=3), undifferentiated connective (n=2), and systemic sclerosis (n=2). Many patients had criteria for several connectivite tissue diseases. Managements were heterogeneous.

Multidimensional analyze identified to groups that were mutually exclusive: (i) Patients characterized by scleroderma-like skin disease,myositis and ILD,frequently treated with high dose of corticosteroids, (ii) Patients with lupus like skin disease, haematological and renal involvements frequently receiving cyclophosphamide and mycophenolate mofetil.

Anti-DNA antibody, absent in the first group and present in the second was the most powerful criteria to distinguish these two subsets of the anti-ku associated disease (sensibility 93% and a sensitivity of 86%).

Conclusions Anti-DNA status distinguishes patients at risk of muscle and lung involvement (anti-DNA negative) from patients at risk of renal involvement (anti-DNA positive). These data significantly refine anti-Ku associated disease and help to personalize monitoring and care of the patients.

Disclosure of Interest None declared

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