Anti-Ku syndrome with elevated CK and anti-Ku syndrome with anti-dsDNA are two distinct entities with different outcomes. Issue 8 (24th May 2019)

• Record Type: Journal Article
• Authors: Spielmann, Lionel; Nespola, Benoit; Séverac, François; Andres, Emmanuel; Kessler, Romain; Guffroy, Aurélien; Poindron, Vincent; Martin, Thierry; Geny, Bernard; Sibilia, Jean; Meyer, Alain
• Is Part Of: Annals of the rheumatic diseases. Volume 78:Issue 8(2019)
• Page Start: 1101
• Page End: 1106
• Publication Date: 2019-05-24
• Subjects: 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; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-214439
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : 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.