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Anti-Ro52 autoantibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis
  1. Sara Sabbagh1,
  2. Iago Pinal-Fernandez1,2,3,
  3. Takayuki Kishi4,
  4. Ira N Targoff5,
  5. Frederick W Miller4,
  6. Lisa G Rider4,
  7. Andrew Lee Mammen1,2,6 The Childhood Myositis Heterogeneity Collaborative Study Group
  1. 1 Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases,National Institutes of Health (NIH), Bethesda, MD, United States
  2. 2 Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, United States
  3. 3 Faculty of Health Sciences, Universitat Oberta de Catalunya, Barcelona, Spain
  4. 4 Environmental Autoimmunity Group, National Institute of EnvironmentalHealth Sciences, National Institutes of Health (NIH), Bethesda, MD, United States
  5. 5 VA Medical Center, Oklahoma Medical Research Foundation, Oklahoma City, OK, United States
  6. 6 Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
  1. Correspondence to Dr Andrew Lee Mammen, NIAMS/NIH, Bethesda, MD 20892, USA; andrew.mammen{at}


Objectives Anti-Ro52 autoantibodies are associated with more severe interstitial lung disease (ILD) in adult myositis patients with antiaminoacyl transfer (t)RNA synthetase autoantibodies. However, few studies have examined anti-Ro52 autoantibodies in juvenile myositis. The purpose of this study was to define the prevalence and clinical features associated with anti-Ro52 autoantibodies in a large cohort of patients with juvenile myositis.

Methods We screened sera from 302 patients with juvenile dermatomyositis (JDM), 25 patients with juvenile polymyositis (JPM) and 44 patients with juvenile connective tissue disease–myositis overlap (JCTM) for anti-Ro52 autoantibodies by ELISA. Clinical characteristics were compared between myositis patients with and without anti-Ro52 autoantibodies.

Results Anti-Ro52 autoantibodies were found in 14% patients with JDM, 12% with JPM and 18% with JCTM. Anti-Ro52 autoantibodies were more frequent in patients with antiaminoacyl tRNA synthetase (64%, p<0.001) and anti-MDA5 (31%, p<0.05) autoantibodies. After controlling for the presence of myositis-specific autoantibodies, anti-Ro52 autoantibodies were associated with the presence of ILD (36% vs 4%, p<0.001). Disease course was more frequently chronic, remission was less common, and an increased number of medications was received in anti-Ro52 positive patients.

Conclusions Anti-Ro52 autoantibodies are present in 14% of patients with juvenile myositis and are strongly associated with anti-MDA5 and antiaminoacyl tRNA synthetase autoantibodies. In all patients with juvenile myositis, those with anti-Ro52 autoantibodies were more likely to have ILD. Furthermore, patients with anti-Ro52 autoantibodies have more severe disease and a poorer prognosis.

  • myositis
  • juvenile idiopathic inflammatory myopathies
  • anti-ro52 autoantibodies
  • myositis associated autoantibodies
  • interstitial lung disease

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  • SS and IP-F are joint first authors.

  • LGR and ALM are joint senior authors.

  • SS and IP-F contributed equally.

  • LGR and ALM contributed equally.

  • Handling editor Josef S Smolen

  • Presented at This work was presented in abstract form at ACR 2018 and GCOM 2019.

  • Collaborators *Members of the Childhood Myositis HeterogeneityCollaborative Study Group who contributed to this project:

    Bita Arabshahi, Lilliana Barillas-Arias, Mara Becker, AprilBingham, Ruy Carrasco, Victoria Cartwright, Rodolfo Curiel, Marietta M.DeGuzman, Barbara Anne Eberhard, Barbara S. Edelheit, Terri Finkel, Stephen W.George, Ellen A. Goldmuntz, William Hannan, Michael Henrickson, Adam M. Huber,Anna Jansen, James Jarvis, Lawrence Jung, Ildy M. Katona, Steven J. Klein, WPatrick Knibbe, Bianca A. Lang, Carol B. Lindsley, Gulnara Mamyrova, LindaMyers, Stephen R. Mitchell, Kabita Nanda, Terrance P. O’Hanlon, Murray H.Passo, Maria D. Perez, Donald A. Person, Linda I. Ray, Rafael F. Rivas-Chacon,Tova Ronis, Deborah Rothman, Adam Schiffenbauer, Bracha Shaham, David Sherry,Abigail Smukler, Matthew L. Stoll, Sangeeta H. Sule, Scott A. Vogelgesang, Rita Volochayev, Jennifer C. Wargula, Pamela Weiss.

  • Contributors SS, IPF, LGR and ALM conceived the work. LGR and INT acquired, analysed and interpreted the data. SS, IPF and TK analysed and interpreted the data. FWM interpreted data. SS, IPF and ALM drafted the work and revised it critically for important intellectual content. TK, IPF, FWM and LGR revised the work for critically important intellectual content. All authors approved the final version of the manuscript. All members of the Childhood Myositis Heterogeneity Collaborative Study Group contributed by (1) providing substantial contributions to the acquisition of data, (2) revising the work critically for important intellectual content and (3) providing final approval of the version published.

  • Funding This research was supported by the Intramural Research Programs of the National Institute of Arthritis and Musculoskeletal and Skin Diseases (ZIA AR041203) and the National Institute of Environmental Health Sciences (Z01 ES101074 and Z01 ES101081) of the National Institutes of Health.

  • Competing interests None declared.

  • Patient and public involvement statement Patients were involved in the research from the time they provided consent to join this natural history study. The research questions were not explicitly developed nor informed by their priorities, experience and preferences. The patients/public were not involved in the design of this study. Patients were not involved in the recruitment to and conduct of the study. Patients were not asked to assess the burden of the intervention and time required to participate in the research. Patients have not and will not be involved in choosing the methods and agreeing plans for dissemination of the study results to participants and wider relevant communities.

  • Patient consent for publication Not required.

  • Ethics approval All subjects were enrolled in natural history study approved by the National Institutes of Health Institutional Institutional Review Board, and all patients provided informed consent.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement All data relevant to the study are included in the article or uploaded as supplementary information.

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