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Canakinumab Use in Periodic Febrile Disorders

Jack Cush, MD

A retrospective records review of patients with periodic fever syndromes (PFS)  receiving IL-1 inhibitor treatment with canakinumab (CAN), shows CAN to be effective and safe in a variety of PFS patients.

Currently CAN is FDA approved to treat the adults and children with cryoprin-associated periodic syndrome (CAPS; including Familial Cold Auto-inflammatory Syndrome [FCAS], Muckle-Wells Syndrome [MWS]), hyperimmunoglobulin D syndrome/mevalonate kinase deficiency, TNF receptor-associated periodic syndrome (TRAPS) and familial Mediterranean fever (FMF), adult-onset Still’s disease (AOSD) and systemic Juvenile Idiopathic Arthritis (SJIA)

A total of 58 US physicians (45% (rheumatologists, 29% allergists/immunologists, 26% dermatologists) participated in a retrospective medical chart review of 147 PFS patients (46% children) with the following PFS:

  • CAPS, 36.7 %;
  • TRAPS, 26.5 %;
  • FMF, 26.5 %;
  • HIDS/MKD, 6.8 %;
  • Mixed, 3.4 %).

Prior to receiving canakinumab, 91% received other agents including NSAIDs (28 %; 40 % in HIDS/MKD); anakinra (24 %; 33% in CAPS); colchicine (22%; 36% in FMF). CAN was started because these treatments for lack of efficacy (39%) or the availability of a new treatment (36%).

Physician reasons for initiating CAN were

  • Perceived efficacy (81%) – highest with HIDS/MKD (91%)
  • Lack of prior treatment response (41%) – highest in in FMF (52%)
  • Good safety profile & tolerability (40%) – More commonly a reason in children compared to adults with PFS
  • Convenience of administration/dosing in CAPS (27%)

Despite canakinumabs use after other antiinflammatory therapies, it was found to be effective, safety and tolerable in a wide variety of PFS subtypes



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