Systemic JIA Foundation

Cycle 1
Web:
Contact:

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 May 2024

Clinical
Disease Class
Rare immunological diseases
Rare systemic and rheumatological diseases of childhood
Body Systems
Hematopoietic / Lymphatic / Immune
Respiratory
Organs
Connective tissue / joints
Heart
Liver
Lungs
Type of Inheritance
Not specified / unknown
Disease Mechanism(s)
Unknown
Age of Onset
Early childhood (age 1+-5)
Incidence
1001-10000
Prevalence
10000+
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
Drugs approved for the disease(s)
· Il-18 inhibitors
Drugs approved for the symptoms
Drugs used off-label
· Jak-inhibitors
Expanded access to drugs
· Emapalumab, mas-825
Organizational & Research
Cell Lines
None
Cell Lines, location
None
Cell Lines, share
N/A
Disease Model
Mouse
Disease Model, location
Children’s Hospital of Philadelphia (CHOP)
Cincinnati Children's Hospital
Disease Model, share
No
Clinical Trial Role
Focus group
Meeting with regulators
Outcome measures, development
Recruitment and outreach, patients
Study material design, review (not protocol)
Biobank
Cincinnati Children's Hospital
Center of Excellence
Cincinnati Children's Hospital
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
Clinical/Treatment Guidelines
No
Organizational Roles
No full-time staff
Science Advisory Board Policies
No policies
Research Network Policies
Has CRN but no policies
Research Roadmap
We don't have a Research Roadmap
International Chapters
None
International Partners
Europe