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

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

Author information

Service de Rhumatologie, hôpitaux civils de Colmar, Colmar, France
Laboratoire d'immunologie, hôpitaux universitaires de Strasbourg, Strasbourg, France.
Service de Santé Publique, GMRC, hôpitaux universitaires de Strasbourg, Strasbourg, France.
ICube, UMR 7357, université de Strasbourg, Strasbourg, France.
Service de médecine interne, hôpitaux universitaires de Strasbourg, Strasbourg, France.
Service de pneumologie, hôpitaux universitaires de Strasbourg, Strasbourg, France.
Service d'immunologie clinique, hôpitaux universitaires de Strasbourg, Strasbourg, France.
Centre de référence national des maladies auto-immunes rares, Strasbourg, France.
Fédération de médecine translationnelle de Strasbourg, FRU 6702, université de Strasbourg, Strasbourg, France.
Service de physiologie et d'explorations fonctionnelles, hôpitaux universitaires de Strasbourg et EA 3072, Strasbourg, France.
Service de rhumatologie, hôpitaux universitaires de Strasbourg, Strasbourg, France.



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


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.


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.


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

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