Clinical Rheumatology

, Volume 37, Issue 4, pp 1011–1015 | Cite as

Predictors of changes in disease activity among children with juvenile dermatomyositis enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry

  • Divya Challa
  • Cynthia S. Crowson
  • Timothy B. Niewold
  • Ann M. ReedEmail author
  • for the CARRA Legacy Registry Investigators
Original Article


Determinants of changes in disease activity among patients with juvenile dermatomyositis (JDM) are unknown. Our objective was to develop predictive models to predict changes in disease activity using the CARRA Legacy Registry. The CARRA Legacy Registry included 658 subjects with definite or probably JDM with 297 subjects with a one follow-up visit after baseline, and we studied the 65 subjects with active disease at baseline. Linear regression models were used to build risk scores for changes in disease activity adjusted for baseline disease activity, age, sex, and disease duration. Disease activity improved from baseline to 6-month follow-up as measured by patient/parent global health score (median 4; p = 0.008), patient pain score (median 2; p = 0.014), physician global (median 4; p < 0.001), and Childhood Myositis Assessment Scale (CMAS) (median 41, p < 0.001). Anti-nuclear antibodies (p = 0.013) and hydroxychloroquine use (p = 0.045) were significant predictors of less improvement in patient/parent global and baseline patient/parent global. Anti-nuclear antibodies (p = 0.001) and V/shawl sign (p = 0.005) were significant predictors of less improvement in patient pain (R-square improved from 0.29 for adjustors alone to 0.46 for the full model). Small joint arthritis (p < 0.01) predicted less improvement and dysphagia/dysphonia (p = 0.033) predicted greater improvement in CMAS and baseline CMAS (R-square improved from 0.73 for adjustors alone to 0.86 for the full model). Disease characteristics can help identify patients who are less likely to improve over time. Risk scores to predict future changes in disease activity could be used to trigger more aggressive treatment earlier in the disease course.


CARRA Disease activity Juvenile dermatomyositis Predictors 



This work could not have been accomplished without the aid of the following organizations: NIAMS and the Arthritis Foundation. We would also like to thank all participants and hospital sites that recruited patients for the CARRA Legacy Registry. The authors thank the following CARRA Registry site principal investigators and research coordinators: L. Abramson, E. Anderson, M. Andrew, N. Battle, M. Becker, H. Benham, T. Beukelman, J. Birmingham, P. Blier, A. Brown, H. Brunner, A. Cabrera, D. Canter, D. Carlton, B. Caruso, L. Ceracchio, E. Chalom, J. Chang, P. Charpentier, K. Clark, J. Dean, F. Dedeoglu, B. Feldman, P. Ferguson, M. Fox, K. Francis, M. Gervasini, D. Goldsmith, G. Gorton, B. Gottlieb, T. Graham, T. Griffin, H. Grosbein, S. Guppy, H. Haftel, D. Helfrich, G. Higgins, A. Hillard, J.R. Hollister, J. Hsu, A. Hudgins, C. Hung, A. Huttenlocher, N. Ilowite, A. Imlay, L. Imundo, C.J. Inman, J. Jaqith, R. Jerath, L. Jung, P. Kahn, A. Kapedani, D. Kingsbury, K. Klein, M. Klein-Gitelman, A. Kunkel, S. Lapidus, S. Layburn, T. Lehman, C. Lindsley, M. Macgregor-Hannah, M. Malloy, C. Mawhorter, D. McCurdy, K. Mims, N. Moorthy, D. Morus, E. Muscal, M. Natter, J. Olson, K. O’Neil, K. Onel, M. Orlando, J. Palmquist, M. Phillips, L. Ponder, S. Prahalad, M. Punaro, D. Puplava, S. Quinn, A. Quintero, C. Rabinovich, A. Reed, C. Reed, S. Ringold, M. Riordan, S. Roberson, A. Robinson, J. Rossette, D. Rothman, D. Russo, N. Ruth, K. Schikler, A. Sestak, B. Shaham, Y. Sherman, M. Simmons, N. Singer, S. Spalding, H. Stapp, R. Syed, E. Thomas, K. Torok, D. Trejo, J. Tress, W. Upton, R. Vehe, E. von Scheven, L. Walters, J. Weiss, P. Weiss, N. Welnick, A. White, J. Woo, J. Wootton, A. Yalcindag, C. Zapp, L. Zemel, and A. Zhu.

Grants/financial supports

This study was funded by a grant from Cure JM and the CARRA Legacy Registry was funded by the National Institute of Arthritis and Musculoskeletal and Skin Diseases, Friends of CARRA, the Arthritis Foundation, and the NIH (RC2AR058934). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

Compliance with ethical standards



Supplementary material

10067_2017_3901_MOESM1_ESM.docx (114 kb)
ESM 1 (DOCX 113 kb)


  1. 1.
    Miller FW, Rider LG, Chung YL, Cooper R, Danko K, Farewell V, Lundberg I, Morrison C, Oakley L, Oakley I et al (2001) Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathies. Rheumatology (Oxford) 40(11):1262–1273CrossRefGoogle Scholar
  2. 2.
    Ruperto N, Ravelli A, Murray KJ, Lovell DJ, Andersson-Gare B, Feldman BM, Garay S, Kuis W, Machado C, Pachman L et al (2003) Preliminary core sets of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis. Rheumatology (Oxford) 42(12):1452–1459CrossRefGoogle Scholar
  3. 3.
    Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (second of two parts). N Engl J Med 292(8):403–407CrossRefPubMedGoogle Scholar
  4. 4.
    Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292(7):344–347CrossRefPubMedGoogle Scholar
  5. 5.
    Feldman BM, Ayling-Campos A, Luy L, Stevens D, Silverman ED, Laxer RM (1995) Measuring disability in juvenile dermatomyositis: validity of the childhood health assessment questionnaire. J Rheumatol 22(2):326–331PubMedGoogle Scholar
  6. 6.
    Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD, Zemel LS, Wallace CA, Ballinger SH, Passo MH et al (2004) Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum 50(5):1595–1603CrossRefPubMedGoogle Scholar
  7. 7.
    Lovell DJ, Lindsley CB, Rennebohm RM, Ballinger SH, Bowyer SL, Giannini EH, Hicks JE, Levinson JE, Mier R, Pachman LM et al (1999) Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies. II. The Childhood Myositis Assessment Scale (CMAS): a quantitative tool for the evaluation of muscle function. The Juvenile Dermatomyositis Disease Activity Collaborative Study Group. Arthritis Rheum 42(10):2213–2219CrossRefPubMedGoogle Scholar
  8. 8.
    Luca NJ, Feldman BM 2013 Disease activity measures in paediatric rheumatic diseases. Int J Rheumatol 715352Google Scholar
  9. 9.
    Sanner H, Sjaastad I, Flato B (2014) Disease activity and prognostic factors in juvenile dermatomyositis: a long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool. Rheumatology 53(9):1578–1585CrossRefPubMedGoogle Scholar
  10. 10.
    Rider LG (2002) Outcome assessment in the adult and juvenile idiopathic inflammatory myopathies. Rheum Dis Clin N Am 28(4):935–977CrossRefGoogle Scholar
  11. 11.
    Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, Barohn RJ, Feldman BM, Harris-Love MO, Koontz DC et al (2013) Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum 65(2):314–324CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Reed AM, Crowson CS, Hein M, de Padilla CL, Olazagasti JM, Aggarwal R, Ascherman DP, Levesque MC, Oddis CV, Group RIMS (2015) Biologic predictors of clinical improvement in rituximab-treated refractory myositis. BMC Musculoskelet Disord 16:257CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2017

Authors and Affiliations

  • Divya Challa
    • 1
  • Cynthia S. Crowson
    • 1
    • 2
  • Timothy B. Niewold
    • 1
    • 3
  • Ann M. Reed
    • 4
    Email author
  • for the CARRA Legacy Registry Investigators
  1. 1.Division of RheumatologyMayo ClinicRochesterUSA
  2. 2.Department of Health Science ResearchMayo ClinicRochesterUSA
  3. 3.Department of ImmunologyMayo ClinicRochesterUSA
  4. 4.Department of PediatricsDuke Children’s Hospital, Duke University Medical CenterDurhamUSA

Personalised recommendations