Canakinumab is a human monoclonal anti-human IL-1β antibody of the IgG1/κ isotype. Canakinumab binds to human IL1β and neutralizes its activity by blocking its interaction with IL-1 receptors, but it does not bind IL-1α or IL-1 receptor antagonist (IL-1ra).

CAPS refer to rare genetic syndromes generally caused by mutations in the NLRP-3 [nucleotide-binding domain, leucine rich family (NLR), pyrin domain containing 3] gene (also known as Cold-Induced Auto-inflammatory Syndrome-1 [CIAS1]). CAPS disorders are inherited in an autosomal dominant pattern with male and female offspring equally affected. Features common to all disorders include fever, urticaria-like rash, arthralgia, myalgia, fatigue, and conjunctivitis.

The NLRP-3 gene encodes the protein cryopyrin, an important component of the inflammasome. Cryopyrin regulates the protease caspase-1 and controls the activation of interleukin-1 beta (IL-1β). Mutations in NLRP-3 result in an overactive inflammasome resulting in excessive release of activated IL-1β that drives inflammation. Systemic juvenile idiopathic arthritis (SJIA) is a severe autoinflammatory disease, driven by innate immunity by means of pro inflammatory cytokines such as interleukin 1β (IL-1β).

ILARIS is an interleukin-1β blocker indicated for the treatment of:

Periodic Fever Syndromes:

• Cryopyrin-Associated Periodic Syndromes (CAPS), in adults and children 4 years of age and older including:
  • Familial Cold Autoinflammatory Syndrome (FCAS) 
  • Muckle-Wells Syndrome (MWS)
• Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) in adult and pediatric patients.
• Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD) in adult and pediatric patients.
• Familial Mediterranean Fever (FMF) in adult and pediatric patients.

Active Systemic Juvenile Idiopathic Arthritis (SJIA) in patients aged 2 years and older.

Administer by subcutaneous injection.

Cryopyrin-Associated Periodic Syndromes (CAPS) 150 mg for CAPS patients with body weight greater than 40 kg and 2 mg/kg for CAPS patients with body weight greater than or equal to 15 kg and less than or equal to 40 kg. For children 15 to 40 kg with an inadequate response, the dose can be increased to 3 mg/kg. Administer subcutaneously every 8 weeks. 

Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS), Hyperimmunoglobulin D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD), and Familial Mediterranean Fever (FMF)

• Body weight less than or equal to 40 kg
  The recommended starting dose is 2 mg/kg every 4 weeks. The dose can be increased to 4 mg/kg every 4 weeks if the clinical response is not adequate.
• Body weight greater than 40 kg
  The recommended starting dose is 150 mg every 4 weeks. The dose can be increased to 300 mg every 4 weeks if the clinical response is not adequate.
• Systemic Juvenile Idiopathic Arthritis (SJIA)
  4 mg/kg (with a maximum of 300 mg) for patients with a body weight greater than or equal to 7.5 kg. Administer subcutaneously every 4 weeks.

CAPS: The most common adverse reactions greater than 10% reported by patients treated with ILARIS are nasopharyngitis, diarrhea, influenza, rhinitis, nausea, headache, bronchitis, gastroenteritis, pharyngitis, weight increased, musculoskeletal pain, and vertigo.

TRAPS, HIDS/MKD, and FMF: The most common adverse reactions greater than 10% reported by patients treated with ILARIS are injection site reactions and nasopharyngitis. 

SJIA: The most common adverse drug reactions greater than 10% reported by patients treated with ILARIS are infections (nasopharyngitis and upper respiratory tract infections), abdominal pain and injection site reactions.

Confirmed hypersensitivity to the active substance or to any of the excipients.

• Interleukin-1 blockade may interfere with immune response to infections. 
  Treatment with medications that work through inhibition of IL-1 has been associated with an increased risk of serious infections. ILARIS has been associated with an increased incidence of serious infections. Physicians should exercise caution when administering ILARIS to patients with infections, a history of recurring infections or underlying conditions which may predispose them to infections. Discontinue treatment with ILARIS if a patient develops a serious infection. Do not administer ILARIS to patients during an active infection requiring medical intervention.
• Live vaccines should not be given concurrently with ILARIS. Prior to initiation of therapy with ILARIS, patients should receive all recommended vaccinations.