FiRst-line Options for Systemic JIA Treatment (FROST) Study

Disease Area:
Juvenile Idiopathic Arthritis (JIA)

Biosample Status:
Biosample Available

About the FROST Study

Systemic juvenile idiopathic arthritis (sJIA) is a rare and aggressive form of childhood arthritis that can cause high fevers, rash, and other symptoms in addition to arthritis. 

There are new and effective treatments for systemic JIA, but we don’t know if using these treatments first makes an important difference in how quickly patients get better, how they do in the long-term, or if they are safer. 

Researchers want to learn:

  • How newly diagnosed sJIA patients do on currently available treatments
  • How effective three different CARRA Consensus Treatment Plans (CTPs), or standard sets of treatment options used in clinical care, are for sJIA

This is one of the first and largest studies that will look at the outcomes of current treatments for patients with newly diagnosed systemic JIA.

Study Updates and Findings

  • Treatment practices have changed greatly since the CARRA sJIA CTPs were initially developed. Initial biologic therapy is now the most common treatment approach among sJIA patients enrolled in the CARRA Registry. 
  • Many patients not initially started on biologics later started a biologic within the first few months following diagnosis.

Read more about the findings: FiRst Line Options for Systemic JIA Treatment (FROST): Results from a Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry Consensus Treatment Plan Observational Study

About the Participants

  • New-onset sJIA patients starting new treatment. Only children between 6 months to 18 years old with an official diagnosis were considered for enrollment. 
  • Fever for at least 2 weeks 
  • Arthritis in 1 or more joints for at least 10 days
  • At least 1 of the following: evanescent rash, generalized lymphadenopathy, hepatomegaly, splenomegaly, or serositis.

About the Study Team

FROST is made possible through support from Genentech and CARRA Registry supporters including the Arthritis Foundation. Blood sample collection is made possible by the Systemic JIA Foundation.

Lead researchers (*Principal Investigator):

  • Yukiko Kimura, MD, Hackensack University Medical Center*
  • Timothy Beukelman, MD MSCE
  • Peter Nigrovic, MD, Brigham and Women’s Hospital
  • Laura Schanberg, MD, Duke Clinical Research Institute and Duke University
  • Marc Natter, MD, Boston Children’s Hospital
  • Brian Feldman, MD, M.Sc., FRCPC, Toronto Hospital for Sick Children

Patient Partners:

  • Vincent Del Gaizo, father of systemic JIA patient
  • Marian Jelenik, young adult with systemic JIA

Related Research Projects

Disease Area:
Juvenile Idiopathic Arthritis (JIA)

CARRA Registry Research Network for SJIA-LD (CARE-NETS) Study

This project launches the first research network to collect clinical data and blood samples from children with systemic JIA and lung disease through the CARRA Registry.

Disease Area:
Juvenile Idiopathic Arthritis (JIA)

Advancing the Science of Pediatric Patient-Reported Outcomes for Children with Chronic Disease (PEPR)

Validation of patient-reported outcomes in chronic diseases.

Disease Area:
Juvenile Idiopathic Arthritis (JIA)

Precision Decisions

Study to develop a biomarker tool to predict response to treatment in polyarticular JIA.