None listed
Conditions
Interventions
Drug : * Treatment:
E4 will be taken by oral once a day, in conjunction with standard of care(SOC).
* Dosing and Schedule :
E4 20 mg/kg will be taken as an initial dose, and may be increased by 5 m
Sponsors
Seoul National University Hospital
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1.Subject Aged 2 to 18 years who meet the criteria for SJIA (ILAR criteria) 2. Subject with an inadequate response, dependence, resistance or side effect to current treatment (NSAIDs and/or corticosteroid) 3. Subject with intermittent fever (body temperature > 38°C) at least 2 days from within 1 week prior to the screening visit and during the screening period 4. Screening laboratory values must meet the following criteria • WBC = 2,500 /µL • Hemoglobin (Hb) = 8.0 g/dL (transfusion permitted) • Platelet = 100,000 /µL • CRP = 1.5 x ULN or ESR = 1.5 x ULN 5. Informed consent provided by the subject (as required by local law), or by the subject's legally authorized representative(s) with the assent of subject who are legally capable of providing it, as applicable
Exclusion criteria
Exclusion criteria: 1. Subject with other autoimmune disease, rheumatoid disease or symptom that overlap other than SJIA 2. History of cancer or lymphoma 3. History of organ or bone marrow transplant surgery 4. Known infection with human immunodeficiency virus (HIV) or acquired immune deficiency syndrome (AIDS) 5. Subject with clinically significant conditions associated with immunodeficiency 6. History of significant cardiac disease caused by SJIA 7. History of severe infectious disease that require hospitalization or treatment within 4 weeks prior to the screening visit 8. Active tuberculosis or history of tuberculosis that require treatment within 2 years prior to the screening visit 9. Subject treated with biological agent(TNF inhibitors, IL-1 inhibitor, IL-6 inhibitor, etc.) within 4 weeks prior to initiation of study treatment 10. Administration of live attenuated vaccine within 4 weeks prior to initiation of study treatment 11. Subject are administrated investigational product in any other type of clinical trial within 4 weeks prior to initiation of study treatment 12. Known hypersensitivity reaction to the study drug ingredients 13. Pregnant or nursing (lactating) female subjects 14. Subject who do not agree to use a proper contraceptive methods
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Number and severity of adverse events evaluated in CTCAE v5.0;Absence of intermittent fever due to SJIA (below 38?);At least 10% of corticosteroid tapering;Change from baseline in WBC and ANC result;Change from baseline in CRP result;Change from baseline in ESR result | — |
Secondary
| Measure | Time frame |
|---|---|
| ACR 30 reponse rate;Improvement of rash due to SJIA;Improvement of hepatosplenomegaly, pleuritis, lymphadenopathy;Period of reccurence | — |
Countries
Korea, Republic of
Contacts
Public ContactSeong Heon Kim
Seoul National University Hospital
Outcome results
None listed