First-line options for systemic juvenile idiopathic arthritis treatment: an observational study of Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans
Authors: Beukelman Timothy , Tomlinson George , Nigrovic Peter A. , Dennos Anne , Del Gaizo Vincent , Jelinek Marian , Riordan Mary Ellen , Schanberg Laura E. , Mohan Shalini , Pfeifer Erin , Kimura Yukiko ,   for the CARRA FROST Investigators, Agbayani R. , Akoghlanian S. , Allenspach E. , Anderson E. , Ardoin S. , Armendariz S. , Balboni I. , Ballenger L. , Ballinger S. , Barbar-Smiley F. , Baszis K. , Bell-Brunson H. , Benham H. , Bernal W. , Bigley T. , Binstadt B. , Blakley M. , Bohnsack J. , Brown A. , Buckley M. , Bullock D. , Cameron B. , Canna S. , Cassidy E. , Chang J. , Chauhan V. , Chinn T. , Chira P. , Cooper A. , Cooper J. , Correll C. , Curiel-Duran L. , Curry M. , Dalrymple A. , De Ranieri D. , Dedeoglu F. , DeGuzman M. , Delnay N. , Dempsey V. , Dowling J. , Drew J. , Driest K. , Du Q. , Durkee D. , Eckert M. , Edens C. , Elder M. , Fadrhonc S. , Favier L. , Feldman B. , Ferguson I. , Ferreira B. , Fogel L. , Fox E. , Fuhlbrigge R. , Fuller J. , George N. , Gerstbacher D. , Gillispie-Taylor M. , Goh I. , Goldsmith D. , Grevich S. , Griffin T. , Guevara M. , Guittar P. , Hager M. , Hahn T. , Halyabar O. , Hance M. , Haro S. , Harris J. , Hausmann J. , Hayward K. , Henderson L. , Hersh A. , Hillyer S. , Hiraki L. , Hiskey M. , Hobday P. , Hoffart C. , Holland M. , Hollander M. , Horwitz M. , Hsu J. , Huber A. , Ibarra M. , Inman C. , Jackson S. , James K. , Janow G. , Jones S. , Jones K. , Jones J. , Justice C. , Khalsa U. , Kienzle B. , Kim S. , Kimura Y. , Kitcharoensakkul M. , Klausmeier T. , Klein K. , Klein-Gitelman M. , Kramer S. , Lai J. , Lang B. , Lapidus S. , Lawson E. , Laxer R. , Lee P. , Lee T. , Lerman M. , Levy D. , Li S. , Lin C. , Ling N. , Lo M. , Lvovich S. , Maller J. , Martyniuk A. , McConnell K. , McHale I. , Meidan E. , Mellins E. , Miller M. , Modica R. , Moore K. , Moussa T. , Mruk V. , Muscal E. , Nanda K. , Nassi L. , Neely J. , Newhall L. , Nigrovic P. , Nolan B. , Oberle E. , Okeke O. , Oliver M. , O’Neil K. , Oz R. , Paller A. , Patel J. , Pepmueller P. , Phillippi K. , Pooni R. , Protopapas S. , Puplava B. , Radhakrishna S. , Ramsey S. , Reid H. , Ringold S. , Riordan M. , Riskalla M. , Ritter M. , Rodriquez M. , Rojas K. , Rosenkranz M. , Rubinstein T. , Sandborg C. , Scalzi L. , Schikler K. , Schmidt K. , Schmitt E. , Schneider R. , Seper C. , Shalen J. , Sheets R. , Shenoi S. , Shirley J. , Silverman E. , Sivaraman V. , Smith C. , Soep J. , Son M. , Spiegel L. , Stapp H. , Stern S. , Stevens A. , Stevens B. , Stewart K. , Stringer E. , Sundel R. , Sutter M. , Syed R. , Syed R. , Tanner T. , Tarshish G. , Tarvin S. , Tesher M. , Thatayatikom A. , Thomas B. , Toib D. , Torok K. , Toruner C. , Tse S. , Valcarcel T. , Vasquez N. , Vehe R. , Velez J. , von Scheven E. , Vora S. , Wagner-Weiner L. , Wahezi D. , Waterfield M. , Weiss P. , Weiss J. , White A. , Woolnough L. , Wright T. , Yee M. , Yeung R. , Yomogida K. , Zhao Y. , Zhu A. 
Published: December 2022

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.