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

Ann Rheum Dis. 2019 Aug;78(8):1101-1106. doi: 10.1136/annrheumdis-2018-214439. Epub 2019 May 24.


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.

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

Publication types

  • Comparative Study

MeSH terms

  • Arthralgia / diagnosis
  • Arthralgia / immunology*
  • Autoantibodies / immunology*
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / immunology*
  • Cluster Analysis
  • Creatine Kinase / blood*
  • DNA-Binding Proteins / metabolism
  • Databases, Factual
  • Female
  • France
  • Glomerulonephritis / diagnosis
  • Glomerulonephritis / immunology*
  • Hospitals, University
  • Humans
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / immunology*
  • Male
  • Syndrome


  • Autoantibodies
  • DNA-Binding Proteins
  • Creatine Kinase