Ilaris (Canakinumab) in the Schnitzler Syndrome
Schnitzler Syndrome
About this trial
This is an interventional treatment trial for Schnitzler Syndrome focused on measuring Schnitzler syndrome
Eligibility Criteria
Inclusion Criteria:
- Patients with active Schnitzler syndrome after withdrawal of anakinra or tapering of corticosteroids.
- Male and female patients at least 18 years of age at the time of screening visit.
- Signed patient informed consent
- Negative QuantiFERON test or negative Purified Protein Derivative (PPD) test (<5 mm induration) at screening or within 1 month prior to the screening visit, according to Belgium guidelines. Patients with a positive PPD test (=5 mm induration) at screeninig may be enrolled only if they have a negative chest x-ray or negative QuantiFERON test (QFT-TB G In-Tube).
- Adequate contraception in females of childbearing potential.
Exclusion Criteria:
- Pregnant or nursing (lactating) women
- History of being immunocompromised, including a positive HIV at screening (ELISA and Western blot)
- Serologic evidence of active hepatitis B or C infection
- Live vaccinations within 3 months prior to the start of the trial, during the trial, and up to 3 months following the last dose.
- History of significant medical conditions, which in the investigator's opinion would exclude the patient from participating in this trial.
- History of recurrent and/or evidence of active bacterial, fungal or viral infection(s).
Sites / Locations
- UZ Gasthuisberg
Arms of the Study
Arm 1
Experimental
Canakinumab
Canakinumab 150 mg (or 2 mg/kg for patients weighing <40kg) every 8 weeks over a 6 months treatment period (i.e., weeks 0, 8, 16 and 24). At Day 7, patients who show an improvement, but not a clinical remission, will be given another 150 mg (or 2 mg/kg for patients weighing <40 kg) injection and continue at 300 mg (or 4 mg/kg for patients weighing <40 kg) every 8 weeks beginning at Week 8. Patients who show no improvement of symptoms and signs of Schnitzler's syndrome will not receive any additional canakinumab dose and will be offered corticosteroid therapy. These patients will return for a follow-up visit 2 weeks later (Day 21) for safety reasons and will be discontinued from the trial. If a patient flares twice during the study, physician may optionally change the dosing frequency to every 4 weeks.