This is a study to evaluate safety and effectiveness of Ilaris in adult and pediatric patients receiving the drug in a clinical setting for any of the following indications, Hereditary Periodic Fever Syndromes, Cryopyrin-associated periodic syndromes (CAPS), colchicine resistance familial Mediterranean fever (crFMF), TNF receptor associated periodic syndrome (TRAPS), Hyper-IgD syndrome / Mevalonate kinase deficiency (HIDS/MKD) or Systemic juvenile idiopathic arthritis (sJIA).
Written informed consent/assent of the patient or their legal representative/parent (≥2 year and <19 year-old pediatric patient) for voluntarily participating in this study
Age: ≥2 year and <19 year-old pediatric and ≥19 year-old adult hereditary periodic fever syndrome (CAPS, crFMF, TRAPS and HIDS/MKD) patients and ≥2 year and <19 year-old sJIA patients
Patient who have an agreement to be treated or who have already started treatment with Ilaris in accordance with the approved label information
Patients receiving Ilaris treatment for autoimmune disease other than CAPS, crFMF, TRAPS, HIDS/MKD or sJIA
Patients participating in an interventional clinical trial which would have an impact on routine clinical treatment
≥2 year and \<19 year-old pediatric and ≥19 year-old adult hereditary periodic fever syndrome patients and ≥2 year and \<19 year-old sJIA patients receiving Ilaris for the treatment of CAPS, crFMF, TRAPS, HIDS/MKD and sJIA
Cohort
Prospective
Ilaris
Biological/VaccineProspective observational study. There is no treatment allocation.
Incidence of adverse events and serious adverse events
Adverse events and serious adverse events in hereditary periodic fever syndrome (CAPS, crFMF, TRAPS and HIDS/MKD) and sJIA patients treated with Ilaris.
Proportion of complete responders
Defining patients as responders when they are assessed as having achieved complete response at 4 weeks of treatment and reach 16 weeks of treatment without flare for crFMF, TRAPS, HIDS/MKD and sJIA patients or complete response at 8 weeks of treatment and reach 16 weeks of treatment without flare for CAPS patients. Complete response will be evaluated in patients with data of clinical response as measured by Physician Global Assessment of Disease Activity (PGA) (5-point scale of none, minor, mild, moderate and severe) Complete response is defined as meeting both of the following criteria. * PGA ≤ minor (PGA \< 2) (clinical response) * CRP ≤10 mg/L (= 1 mg/dL), or reduction by ≥70% from the start of treatment (serological response) Flare is defined as meeting both of the following criteria. * PGA ≥ mild (PGA ≥ 2) (clinical flare) * CRP \>30 mg/L (= 3 mg/dL) (serological flare)
Proportion of participants with Physician Global Assessment of Disease Activity (PGA) score <2
Participants assessed by physician on Physician's Global Assessment measured on a 5--point scale for disease activity as: 0 = None/absent; 1 = Minor; 2 = Mild; 3 = Moderate; 4 = Severe.
Proportion of patients with C- reactive protein (CRP) serological response
Proportion of patients with a C-reactive protein (CRP) of ≤ 10 mg/L or a ≥ 70% decrease from the start of treatment.
Proportions of patients with serum amyloid A (SAA) normalization
Proportions of patients with serum amyloid A (SAA) normalization defined as SAA ≤ 10 mg/L.
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