First-line options for systemic juvenile idiopathic arthritis treatment: an observational study of Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans

for the CARRA FROST Investigators

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5 Scopus citations


Background: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans (CTPs) to compare treatment initiation strategies for systemic juvenile idiopathic arthritis (sJIA). First-line options for sJIA treatment (FROST) was a prospective observational study to assess CTP outcomes using the CARRA Registry. Methods: Patients with new-onset sJIA were enrolled if they received initial treatment according to the biologic CTPs (IL-1 or IL-6 inhibitor) or non-biologic CTPs (glucocorticoid (GC) monotherapy or methotrexate). CTPs could be used with or without systemic GC. Primary outcome was achievement of clinical inactive disease (CID) at 9 months without current use of GC. Due to the small numbers of patients in the non-biologic CTPs, no statistical comparisons were made between the CTPs. Results: Seventy-three patients were enrolled: 63 (86%) in the biologic CTPs and 10 (14%) in the non-biologic CTPs. CTP choice appeared to be strongly influenced by physician preference. During the first month of follow-up, oral GC use was observed in 54% of biologic CTP patients and 90% of non-biologic CTPs patients. Five (50%) non-biologic CTP patients subsequently received biologics within 4 months of follow-up. Overall, 30/53 (57%) of patients achieved CID at 9 months without current GC use. Conclusion: Nearly all patients received treatment with biologics during the study period, and 46% of biologic CTP patients did not receive oral GC within the first month of treatment. The majority of patients had favorable short-term clinical outcomes. Increased use of biologics and decreased use of GC may lead to improved outcomes in sJIA.

Original languageEnglish (US)
Article number113
JournalPediatric Rheumatology
Issue number1
StatePublished - Dec 2022

Bibliographical note

Funding Information:
TB has received consulting fees from Novartis and UCB. PAN receives investigator-initiated research grants from Bristol-Myers Squibb and Pfizer; consulting from Bristol-Myers Squibb, Cerecor, Exo Therapeutics, Miach Orthopedics, Novartis, and Pfizer; royalties from UpToDate Inc.; and salary support from the Childhood Arthritis and Rheumatology Research Alliance. LES has received research support from Bristol-Myers Squibb. LES serves on the data and safety monitoring board for Sanofi (sarilumab) and UCB (certolizumab). SM and EP are employees and shareholders of Genentech, Inc. YK has received research support from Genentech.

Funding Information:
Funding for this project was provided to CARRA, Inc. in part by Genentech, a member of the Roche Group.

Funding Information:
This work could not have been accomplished without the aid of the following organizations: The NIH’s National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) & the Arthritis Foundation. We would also like to thank all participants and hospital sites that recruited patients for the CARRA Registry. The authors thank the following CARRA Registry site principal investigators, sub-investigators, and research coordinators: R. Agbayani, S. Akoghlanian, E. Allenspach, E. Anderson, S. Ardoin, S. Armendariz, I. Balboni, L. Ballenger, S. Ballinger, F. Barbar-Smiley, K. Baszis, H. Bell-Brunson, H. Benham, W. Bernal, T. Bigley, B. Binstadt, M. Blakley, J. Bohnsack, A. Brown, M. Buckley, D. Bullock, B. Cameron, S. Canna, E. Cassidy, J. Chang, V. Chauhan, T. Chinn, P. Chira, A. Cooper, J. Cooper, C. Correll, L. Curiel-Duran, M. Curry, A. Dalrymple, D. De Ranieri, F. Dedeoglu, M. DeGuzman, N. Delnay, V. Dempsey, J. Dowling, J. Drew, K. Driest, Q. Du, D. Durkee, M. Eckert, C. Edens, M. Elder, S. Fadrhonc, L. Favier, B. Feldman, I. Ferguson, B. Ferreira, L. Fogel, E. Fox, R. Fuhlbrigge, J. Fuller, N. George, D. Gerstbacher, M. Gillispie-Taylor, I. Goh, D. Goldsmith, S. Grevich, T. Griffin, M. Guevara, P. Guittar, M. Hager, T. Hahn, O. Halyabar, M. Hance, S. Haro, J. Harris, J. Hausmann, K. Hayward, L. Henderson, A. Hersh, S. Hillyer, L. Hiraki, M. Hiskey, P. Hobday, C. Hoffart, M. Holland, M. Hollander, M. Horwitz, J. Hsu, A. Huber, M. Ibarra, C. Inman, S. Jackson, K. James, G. Janow, S. Jones, K. Jones, J. Jones, C. Justice, U. Khalsa, B. Kienzle, S. Kim, Y. Kimura, M. Kitcharoensakkul, T. Klausmeier, K. Klein, M. Klein-Gitelman, S. Kramer, J. Lai, B. Lang, S. Lapidus, E. Lawson, R. Laxer, P. Lee, T. Lee, M. Lerman, D. Levy, S. Li, C. Lin, N. Ling, M. Lo, S. Lvovich, J. Maller, A. Martyniuk, K. McConnell, I. McHale, E. Meidan, E. Mellins, M. Miller, R. Modica, K. Moore, T. Moussa, V. Mruk, E. Muscal, K. Nanda, L. Nassi, J. Neely, L. Newhall, P. Nigrovic, B. Nolan, E. Oberle, O. Okeke, M. Oliver, K. O’Neil, R. Oz, A. Paller, J. Patel, P. Pepmueller, K. Phillippi, R. Pooni, S. Protopapas, B. Puplava, S. Radhakrishna, S. Ramsey, H. Reid, S. Ringold, M. Riordan, M. Riskalla, M. Ritter, M. Rodriquez, K. Rojas, M. Rosenkranz, T. Rubinstein, C. Sandborg, L. Scalzi, K. Schikler, K. Schmidt, E. Schmitt, R. Schneider, C. Seper, J. Shalen, R. Sheets, S. Shenoi, J. Shirley, E. Silverman, V. Sivaraman, C. Smith, J. Soep, M. Son, L. Spiegel, H. Stapp, S. Stern, A. Stevens, B. Stevens, K. Stewart, E. Stringer, R. Sundel, M. Sutter, R. Syed, R. Syed, T. Tanner, G. Tarshish, S. Tarvin, M. Tesher, A. Thatayatikom, B. Thomas, D. Toib, K. Torok, C. Toruner, S. Tse, T. Valcarcel, N. Vasquez, R. Vehe, J. Velez, E. von Scheven, S. Vora, L. Wagner-Weiner, D. Wahezi, M. Waterfield, P. Weiss, J. Weiss, A. White, L. Woolnough, T. Wright, M. Yee, R. Yeung, K. Yomogida, Y. Zhao, A. Zhu.

Funding Information:
TB is supported by CARRA. PAN is funded by NIAMS awards 2R01AR065538 and R01AR073201. LES is supported by the NIAMS award number U19AR069522, the Patient-Centered Outcomes Research Institute under award number PaCr-2017C2-8177, and the CARRA. YK is supported by CARRA.

Publisher Copyright:
© 2022, The Author(s).


  • Biologics
  • Juvenile idiopathic arthritis
  • Still’s disease
  • Systemic juvenile idiopathic arthritis
  • Treatment


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