Repsonse to Treatment with Canakinumab without steroids early after diagnosis of systemic onset juvenile idiopathic arthritis / juvenile Still’s disease
- Conditions
- systemic juvenile idiopathic arthritisMedDRA version: 20.0Level: LLTClassification code 10079454Term: Systemic juvenile idiopathic arthritisSystem Organ Class: 100000004859Therapeutic area: Diseases [C] - Immune System Diseases [C20]
- Registration Number
- EUCTR2018-004284-30-DE
- Lead Sponsor
- Asklepios Klink Sankt Augustin
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Authorised-recruitment may be ongoing or finished
- Sex
- All
- Target Recruitment
- 20
Parents / legal guardian and patient are willing to participate in the study and signed voluntarily the Informed Consent form.
?I 2 Patient and parents / legal guardian agree to comply with study requirements and are able to be at the clinic for all required study visits.
?I 3Patient is at least 2 years old and has not reached his 18th birthday.
?I 4Patient is currently not treated with a disease-modifying antirheumatic drug (DMARD). Patient has not been treated with synthetic DMARDs (such as Methotrexate, Leflunomide, Azathioprine, Hydroxychloroquine, Chloroquine …) or biologic DMARDs (such as Anakinra, Canakinumab, Tocilizumab, Etanercept, Adalimumab, Golimumab …) before baseline.
?I 5Stable dosage of NSAIDs
?I 6Patient is currently not treated with corticosteroids. Patient has previously not been treated with corticosteroids for more than 7 days after diagnosis.
?I 7IN FEMALE PATIENT IN WHOM MENARCHE HAS OCCURRED
•Negative serum pregnancy test prior to administration of study medication.
•Willingness to use an adequate method of contraception
Adequate contraception can include abstinence if the investigator deems appropriate.
? I 8Diagnosis of active systemic onset JIA as determined by International League of Associations for Rheumatology (ILAR) criteria (Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, et al. J Rheumatol. 2004;31:390–392) or the Yamaguchi’s criteria for Still’disease ( Yamaguchi M. et al., J Rheumatol. 19:424-30, 1992) with confirmed diagnosis of SJIA as per ILAR definition:
Arthritis in one or more joints with or preceded by fever of at least 2 weeks duration that is documented to be daily/ quotidian for at least 3 days and accompanied by one or more of the following:
· Evanescent nonfixed erythematous rash,
· Generalized lymph node enlargement,
· Hepatomegaly and/ or splenomegaly,
· Serositis
or
confirmed diagnosis of Still’s disease as per Yamaguchi’s criteria:
Five or more criteria, of whom two or more must be major
Major criteria
? Fever >39 °C, lasting 1 week or longer
? Arthralgia or arthritis, lasting 2 weeks or longer
? Typical rash
? Leukocytosis >10,000/mm3 with >80% polymorphonuclear cells
Minor criteria
? Sore throat
? Recent development of significant lymphadenopathy
? Hepatomegaly or splenomegaly
? Abnormal liver function tests
? Negative tests for antinuclear antibody (IF) and rheumatoid factor (IgM)
Exclusion criteria
? Infections
? Malignancies (mainly malignant lymphoma)
? Other rheumatic disease (mainly systemic vasculitides)
The activity of the disease is judged with
(I)active arthritis joints with either swelling not due to deformity or if no
swelling is present with limiting of motion and pain or pain on movement,
(II)a least a score of 3 of 10 for global assessment of the severity of disease by the physician
(III)a least a score of 3 of 10 for global assessment of overall well-being by the
patient or parent
(IV)active systemic signs of the disease for at least 3 days before baseline with either fever of more than 38.5°C, and/or pericarditis and/or rash.
?I 9Patient have to meet all criteria for eligibility for treatment with Canakinumab according to SPC and local guidelines, with exception of the requirement of a minimum of five affected joints.
?I 10Either the subject or an available adult must be capable (according to the investigator´s judgment of reconstitu
E 1Chronic or active infection or any major episode of infection requiring hospitalization or treatment with intravenous antibiotics within 30 days prior to study screening
E 2Any preceding diagnosis of malignancy.
E 3Pregnant or breast feeding female.
E 4Female not willing to use appropriate contraception or sexual abstinence.
E 5Active gastrointestinal disease (e.g., inflammatory bowel disease)
E 6Significant blood clotting defect
E 7Patient has a history of any chronic disease other than JIA, especially chronic renal disease, liver disease, hematological, gastrointestinal, pulmonary, cardiological or neurological disease, which in the opinion of the investigator may influence the efficacy or safety of the study medication or which in the opinion of the investigator leads to an unacceptable risk for the patient if he participates in the Study.
E 8Patient had a significant illness during a period of 4 weeks prior to the first administration of study medication other than JIA-related.
E 9Previous use of systemic corticosteroids for more than 7 days (>0.2 mg/kg bw)
E10Previous use of immunosuppressants (such as methotrexate, cyclosporine)
E11Previous use of biologic agents (such as Anakinra, Canakinumab, Tocilizumab)
E12Active macrophage activation syndrome with biologic features of MAS [such as hemorrhages, central nervous system dysfunction, plasma fibrinogen level < 2.5 g/L, cytopenia, hypertriglyceridemia, decreased platelet count, hyperferritinemia at screening or a history of recurrent pericarditis, myocarditis, serositis.
E 13AST (SGOT), ALT (SGPT), or BUN levels more than two times the upper level of normal, creatinine levels more than 1.5 mg/dl, or any other laboratory abnormality considered to be clinically significant within 28 days prior to baseline
E 14Received any investigational medication within 30 days prior to the first dose of study medication or scheduled to receive an investigational drug (other than the study medications) during the course of the study
E 15Patient has previously been admitted to this study.
E 16Patients is known to be HIV-infected
E 17Known past or current hepatitis infection
E 18Patient has a history of an expanding CNS neoplasm, active CNS infection, demyelinating disease, degenerative neurological disease or any progressive CNS disease.
E 19Patient has a poorly controlled diabetes.
E 20Received a live virus vaccine within 1 month prior to baseline
E 21Any concurrent medical condition which would, in the investigator's opinion, compromise the patient's ability to tolerate the study drug or would make the patient unable to comply with the protocol.
E 22Patient has a history of or current psychiatric illness that would interfere with ability to comply with protocol requirements or give informed consent.
E 23Patient has a recent history of alcohol or drug abuse within the past 6 months that would interfere with ability to comply with protocol requirements.
E 24Any contraindication listed in the German 'Fachinformation' of the drug Ilaris ®
Study & Design
- Study Type
- Interventional clinical trial of medicinal product
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method
- Secondary Outcome Measures
Name Time Method