Canakinumab in Patients With Active Hyper-IgD Syndrome
Canakinumab in Patients With Active Hyper-IgD Syndrome
This pilot study is designed to evaluate the efficacy, the safety, and the pharmacokinetics
 (PK) / pharmacodynamics (PD) of canakinumab treatment in patients with HIDS.
Inclusion Criteria:

 1. Patients with a diagnosis of HIDS proven by DNA analysis and/or enzymatic studies.

 2. At time of start of drug treatment: active HIDS as evidenced by a physician global
 assessment of HIDS flare severity ≥ 2 and CRP values >10 mg/L (normal CRP < or = 10
 mg/L).

 3. Patients who have a history of > or = 3 febrile acute HIDS flares in a 6-month period
 when not receiving prophylaxis treatment (e.g. anakinra daily treatment) with a
 duration of each flare lasting > or = 4 days and limiting the normal daily activities.

 Exclusion Criteria:

 1. Pregnant or nursing (lactating) women.

 2. History of being immunocompromised, including a positive HIV at screening (ELISA and
 Western blot) test result.

 3. Positive Hepatitis B or Hepatitis C.

 4. Live vaccinations within 3 months prior to the start of the trial

 5. Positive tuberculosis screening test.

 Other protocol-defined inclusion/exclusion criteria may apply