Systemic JIA Foundation

Cycle 1

Systemic juvenile idiopathic arthritis (JIA) is a are pediatric rheumatological disease characterized by the variable occurrence of chronic arthritis, intermittently high spiking fever, maculopapular rash during fever episodes, hepatomegaly and/or splenomegaly, lymphadenopathy, and serositis.

Last updated 04/30/2025

Clinical
Disease Class
Immunological diseases
Systemic and rheumatological diseases of childhood
Body Systems
Hematopoietic / Lymphatic / Immune
Respiratory
Organs
Connective tissue / joints
Heart
Liver
Lungs
Known Genetic Link
Yes, genetic factors contribute to the risk or severity of the condition
causative_genes
None specified / unknown
contributory_genes
None specified / unknown
Type of Inheritance
Not specified / unknown
Newborn Screening
Not applicable
Disease Mechanism(s)
Aberrant immune response
Age of Onset
Early childhood (age 1+-5)
Average Age at Diagnosis
Early childhood (age 1+-5)
Life Expectancy
Adulthood (age 18-64)
Affected Sex(es)
Female
Male
National Prevalence
10000+
Global Prevalence
Unknown
National Incidence
11-50
Global Incidence
Unknown
Populations and/or ancestry with higher prevalence
More cases than typical in India and Japan
Symptoms / Phenotypes
fever
joint pain / arthralgia
skin redness and/or swelling
Biomarkers
Diagnostic
· Il18 level, Interferon gamma markers such as cxcl9
Monitoring
· Ferritin, crp, cbc, platelets, il18
Therapeutic
· Il18, cxcl9
Existing Therapies
Expanded access (Compassionate Use)
FDA-Approved for Symptom Relief
· Biologics, jak inhibitors
FDA-Approved to Cure or Modify the Disease
Off-Label Drug Use
Organizational & Research
Cell Lines
None
Cell Lines, Institution
None
Cell Lines, share
N/A
Disease Model
None
Disease Model, share
N/A
Clinical Trial Role
Focus group
Meeting with regulators
Outcome measures, development
Recruitment and outreach, patients
Study material design, review (not protocol)
Biobank, Institution
Cincinnati Children's Hospital
Biobank, Involvement
Consulted
Funded
Center of Excellence, Institution
Children’s Hospital of Philadelphia (CHOP)
Cincinnati Children's Hospital
Center of Excellence, Involvement
Consulted
Funded
Registry
Yes, we have collaborated on a registry
Data Collected, Registry
Medication usage
Patient contact info
Patient-reported data
Data Entered by, Registry
Patients
Platform, Registry
Not specified
Natural History Study
Yes, we have collaborated on a natural history study
Data Collected, Natural History Study
Electronic health records/electronic medical records
Medication usage
Patient-reported outcomes
Prospective data
Retrospective data
Platform, Natural History Study
IQVIA
REDCap
FDA Patient Listening Session
Yes
FDA Patient-Focused Drug Development (PFDD) Program
No
ICD Codes
Yes, we have an ICD-11 code specific to our exact disease
Diagnostic Guidelines
No
Science Advisory Board Policies
No policies
Research Network Policies
Has CRN but no policies
Research Roadmap
Yes we have a Research Roadmap, but will not share policies
International Chapters
None
International Partners
Europe
South America