Article Text

Download PDFPDF
Anti-Ku syndrome with elevated CK and anti-Ku syndrome with anti-dsDNA are two distinct entities with different outcomes


Objective To refine the spectrum of anti-Ku-associated disease, a condition that is equivocally described by current diagnostic criteria for connective tissue diseases.

Methods Among 42 consecutive patients harbouring anti-Ku antibodies, subgroups with similar phenotypes and prognosis were delineated without an a priori diagnosis using hierarchical clustering analysis of the cumulative clinico-biological features recorded during the follow-up. Features present at baseline that most efficiently predicted the outcomes were then identified using a sensitivity–specificity sum maximisation approach.

Results Clinico-biological features were clustered into three groups. Glomerulonephritis and ILD, the two fatal complications in this cohort, were unequally distributed between the three clusters that additionally differed on six clinico-biological features.

Among features present at baseline, elevated serum level of creatine kinase (CK) and anti-dsDNA antibodies were generally mutually exclusive and most efficiently predicted the cluster belonging at last follow-up. Anti-Ku patients with elevated CK had a 22-fold higher risk of ILD while anti-Ku patients with anti-dsDNA antibodies had a 13-fold higher risk of glomerulonephritis

Conclusion “Anti-Ku with elevated CK” syndrome and “anti-Ku with anti-dsDNA” syndrome represent two distinct entities that are important to recognise in order to best tailor patient care.

  • autoantibodies
  • autoimmune diseases
  • autoimmunity
  • Dermatomyositis
  • Polymyositis
  • Inflammatory myopathies
  • Inflammatory myopathy
  • Myositis
  • Necrotizing myopathy
  • necrotizing myopathies
  • Inflammatory skeletal muscle
  • Antisynthetase
  • Interstitial lung disease
  • Systemic lupus erythematosus
  • Systemic sclerosis
  • scleroderma
  • Classification
  • Anti-Ku antibodies
  • Undifferentiated connective tissue disease
  • Mixed connective tissue disease

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.