Anti-NT5C1A autoantibodies are associated with more severe disease in patients with juvenile myositis. Issue 5 (23rd January 2018)

• Record Type: Journal Article
• Authors: Yeker, Richard M; Pinal-Fernandez, Iago; Kishi, Takayuki; Pak, Katherine; Targoff, Ira N; Miller, Frederick W; Rider, Lisa G; Mammen, Andrew L
• Other Names: author non-byline.; Albert Daniel A author non-byline.; Arabshahi Bita author non-byline.; Balboni Imelda M author non-byline.; Bohnsack John F author non-byline.; Borzy Michael S author non-byline.; Bowyer (postmortem) Suzanne L author non-byline.; T chao Chun Peng author non-byline.; Cron Randy Q author non-byline.; Deguzman Marietta M author non-byline.; Goldmuntz Ellen A author non-byline.; Griffin Thomas A author non-byline.; Hannan William author non-byline.; Hawkins-holt Melissa author non-byline.; Hollister J Roger author non-byline.; Hopp Russell J author non-byline.; Jacobs (postmortem) Jerry C author non-byline.; Jansen Anna author non-byline.; Jones Olcay Y author non-byline.; Jung Lawrence K author non-byline.; Kamdar Ankur author non-byline.; Klein Steven J author non-byline.; Lang Bianca A author non-byline.; Madson Katherine L author non-byline.; Mccarthy Paul L author non-byline.; Mamyrova Gulnara author non-byline.; Mitchell Stephen R author non-byline.; Murphy Frederick T author non-byline.; Nanda Kabita author non-byline.; O'hanlon Terrance P author non-byline.; Oral Elif A author non-byline.; Pachman Lauren M author non-byline.; Passo Murray H author non-byline.; Perez Maria D author non-byline.; Person Donald A author non-byline.; Ray Linda I author non-byline.; Rennebohm Robert M author non-byline.; Rivas-chacon Rafael F author non-byline.; Ronis Tova author non-byline.; Schiffenbauer Adam author non-byline.; Shaham Bracha author non-byline.; Sherry David D author non-byline.; Sinal Sara H author non-byline.; Smukler Abigail author non-byline.; Sule Sangeeta H author non-byline.; Vogelgesang Scott A author non-byline.; Wargula Jennifer C author non-byline.; White Patience H author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 77:Issue 5(2018)
• Page Start: 714
• Page End: 719
• Publication Date: 2018-01-23
• Subjects: dermatomyositis -- polymyositis -- autoantibodies -- autoimmunity -- juvenile idiopathic arthritis; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2017-212436
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: Autoantibodies recognising cytosolic 5′-nucleotidase 1A (NT5C1A) are found in adult patients with myositis and other autoimmune diseases. They are especially prevalent in adults with inclusion body myositis (IBM), in which they are associated with more severe weakness and higher mortality. This study was undertaken to define the prevalence and clinical features associated with anti-NT5C1A autoantibodies in juvenile myositis. Methods: We screened sera from 380 patients with juvenile myositis, 30 patients with juvenile idiopathic arthritis (JIA) and 92 healthy control children for anti-NT5C1A autoantibodies. Clinical characteristics were compared between patients with myositis with and without anti-NT5C1A autoantibodies. Results: Anti-NT5C1A autoantibodies were present in 102 of 380 (27%) patients with juvenile myositis and in 11 of 92 (12%) healthy control children (P=0.002) and 27% of children with JIA (P=0.05 vs controls). Sera of 83 of 307 (27%) patients with juvenile dermatomyositis and 16 of 46 (35%) patients with juvenile overlap myositis were anti-NT5C1A autoantibody-positive (P<0.01 vs healthy controls for each), but sera of only 3 of 27 (11%) patients with juvenile polymyositis were anti-NT5C1A-positive. Patients with juvenile myositis with and without anti-NT5C1A autoantibodies had similar clinical phenotypes. However, patients with anti-NT5C1A autoantibody-positive myositis had greater pulmonary symptoms at diagnosis (P=0.005), more frequent hospitalisations (P=0.01) and required a larger number of medications (P<0.001). Conclusion: Anti-NT5C1A autoantibodies are present in more than one-quarter of children with juvenile myositis and JIA compared with only 12% of healthy children, suggesting they are myositis-associated in children. As in adults with IBM, patients with juvenile myositis with anti-NT5C1A autoantibodies have more severe disease.