Prescribing information



Key safety profile data for ILARIS in the treatment of familial Mediterranean fever (FMF), hyperimmunoglobulin D syndrome (HIDS)/mevalonate kinase deficiency (MKD), tumour necrosis factor receptor-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndromes (CAPS).

Please refer to the ILARIS Summary of Product Characteristics (SmPC) for full safety information.1

  • The most frequent adverse drug reactions were infections predominantly of the upper respiratory tract. No impact on the type or frequency of adverse drug reactions was seen with longer-term treatment.1
  • Hypersensitivity reactions have been reported in patients treated with canakinumab.1
  • Opportunistic infections have been reported in patients treated with canakinumab (please see ‘Special warnings and precautions for use’ below).1

Tabulated list of adverse reactions

MedDRA System

Organ Class

Infections and infestations
Very common Respiratory tract infections (including pneumonia, bronchitis, influenza, viral infection, sinusitis, rhinitis, pharyngitis, tonsillitis, nasopharyngitis, upper respiratory tract infection)

Ear infection



Urinary tract infection
Common Vuvovaginal candidiasis
Nervous system disorders
Common Dizziness/vertigo
Gastrointestinal disorders
Very common Upper abdominal pain*
Skin and subcutaneous tissue disorders
Very common Injection site reaction
Musculoskeletal and connective tissue disorders
Very common Arthralgia*
Common Musculoskeletal pain*
Very common Creatinine renal clearance decreased*,†


Leukopenia* (see ‘Laboratory anomalies’ below)
Common Neutropenia (see ‘Laboratory anomalies’ below)
Uncommon Platelet count decreased (see ‘Laboratory anomalies’ below)

Adapted from ILARIS® (canakinumab) Summary of Product Characteristics.1

Laboratory anomalies1

Long-term data and laboratory abnormalities in CAPS patients

During clinical trials with canakinumab in CAPS patients mean values for haemoglobin increased, and those for white blood cell, neutrophils and platelets decreased.

Elevations of transaminases have been observed rarely in CAPS patients.

Asymptomatic and mild elevations of serum bilirubin have been observed in CAPS patients treated with canakinumab without concomitant elevations of transaminases.

In the long-term, open-label studies with dose escalation, events of infections (gastroenteritis, respiratory tract infection, upper respiratory tract infection), vomiting and dizziness were more frequently reported in the 600 mg or 8 mg/kg dose group than in other dose groups.

Laboratory abnormalities in TRAPS, HIDS/MKD and FMF patients

Neutrophils: Although ≥ Grade 2 reductions in neutrophil count occurred in 6.5% of patients (common) and Grade 1 reductions occurred in 9.5% of patients, the reductions are generally transient and neutropenia-associated infection has not been identified as an adverse reaction.

Platelets: Although reductions in platelet count (≥ Grade 2) occurred in 0.6% of patients, bleeding has not been identified as an adverse reaction. Mild and transient Grade 1 reduction in platelets occurred in 15.9% of patients without any associated bleeding adverse events.

Special warnings and precautions for use1


Canakinumab is associated with an increased incidence of serious infections. Therefore patients should be monitored carefully for signs and symptoms of infections during and after treatment with canakinumab. Physicians should exercise caution when administering canakinumab to patients with infections, a history of recurring infections, or underlying conditions which may predispose them to infections.

Canakinumab should not be initiated or continued in patients during an active infection requiring medical intervention.

Tuberculosis screening

In approximately 12% of CAPS patients tested with a PPD (purified protein derivative) skin test in clinical trials, follow-up testing yielded a positive test result while treated with canakinumab without clinical evidence of a latent or active tuberculosis infection. It is unknown whether the use of interleukin-1 (IL-1) inhibitors such as canakinumab increases the risk of reactivation of tuberculosis. Before initiation of therapy, all patients must be evaluated for both active and latent tuberculosis infection.

Neutropenia and leukopenia

Neutropenia (absolute neutrophil count [ANC] <1.5 x 109/l) and leukopenia have been observed with medicinal products that inhibit IL-1, including canakinumab. Treatment with canakinumab should not be initiated in patients with neutropenia or leukopenia. It is recommended that white blood cell (WBC) counts including neutrophil counts be assessed prior to initiating treatment and again after 1 to 2 months.


Malignancy events have been reported in patients treated with canakinumab. The risk for the development of malignancies with anti-interleukin (IL)-1 therapy is unknown.

Hypersensitivity reactions

Hypersensitivity reactions with canakinumab therapy have been reported. The majority of these events were mild in severity. During clinical development of canakinumab in over 2,600 patients, no anaphylactoid or anaphylactic reactions attributable to treatment with canakinumab were reported. However, the risk of severe hypersensitivity reactions, which is not uncommon for injectable proteins, cannot be excluded.

Hepatic function

Transient and asymptomatic cases of elevations of serum transaminases or bilirubin have been reported in clinical trials.


No data are available on the risk of secondary transmission of infection by live (attenuated) vaccines in patients receiving canakinumab. Therefore, live vaccines should not be given concurrently with canakinumab unless the benefits clearly outweigh the risks.

Mutation in NLRP3 gene in CAPS patients

Clinical experience in CAPS patients without a confirmed mutation in the NLRP3 gene is limited.

Please refer to the SmPC for more information and the full list of special warnings and precautions for use.1

  • In the β-Confident Registry, the largest documented CAPS cohort, ILARIS demonstrated a safety profile consistent with its clinical trial programme up to 5 years2
  • In general, the incidence of adverse events in each dose group of ILARIS increased with age (<4 to <65 years)3
  • An increase in the dose of ILARIS from 2–<4 mg/kg to 4–<8 mg/kg was not associated with an increased incidence of adverse events in any age group3

5-year safety profile of ILARIS in the β-Confident Registry§2

  No. of patients

Incidence rate per

100 patient-years
Overall adverse events   100.0
Infections and infestations   36.7
Vertigo 19 (6.6%) 3.7
Serious adverse events 62 (21.5%) 15.0
Serious infections (see FMF, HIDS/MKD, TRAPS and CAPS: Special warnings and precautions for use)   4.1
Discontinuation due to adverse event, poor efficacy and patient preference 21 (7.3%)  
Discontinuation due to adverse events 5 (1.7%)  
Deaths 1 (0.3%)  

Adapted from Kuemmerle-Deschner et al. 2015.2


Periodic fever syndromes

ILARIS is indicated for the treatment of the following autoinflammatory periodic fever syndromes in adults, adolescents and children aged 2 years and older:

  • CAPS, including:
    • Muckle-Wells syndrome (MWS)
    • Neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurological, cutaneous, articular syndrome (CINCA)
    • Severe forms of familial cold autoinflammatory syndrome (FCAS)/familial cold urticaria (FCU) presenting with signs and symptoms beyond cold-induced urticarial skin rash
  • FMF
    • ILARIS should be given in combination with colchicine, if appropriate.

Still’s disease

ILARIS is indicated for the treatment of active Still’s disease including adult-onset Still’s disease (AOSD) and systemic juvenile idiopathic arthritis (SJIA) in patients aged 2 years and older who have responded inadequately to previous therapy with non-steroidal anti-inflammatory drugs (NSAIDs) and systemic corticosteroids. ILARIS can be given as monotherapy or in combination with methotrexate.

Based on estimated creatinine clearance, most were transient.
Most represented transient trace to 1+ positive urinary protein by dipstick.
§288 patients were enrolled with a mean±SD duration of 193±72 weeks.2
One death was reported due to metastatic rectal adenocarcinoma in a 76-year-old patient.2

ANC, absolute neutrophil count; CAPS, cryopyrin-associated periodic syndromes; CINCA, chronic infantile neurological, cutaneous, articular syndrome; FCAS, familial cold autoinflammatory syndrome; FCU, familial cold urticaria; FMF, familial Mediterranean fever; HIDS, hyperimmunoglobulin D syndrome; MKD, mevalonate kinase deficiency; MWS, Muckle-Wells syndrome; NOMID, neonatal-onset multisystem inflammatory disease; NSAID, non-steroidal anti-inflammatory drug; SJIA, systemic juvenile idiopathic arthritis; TRAPS, tumour necrosis factor receptor-associated periodic syndrome; WBC, white blood cell count.


  1. ILARIS® (canakinumab) Summary of Product Characteristics.
  2. Kuemmerle-Deschner JB et al. Pediatr Rheumatol 2015;13(Suppl 1):P3.
  3. Kuemmerle-Deschner JB et al. Abstract 1411 presented at 2018 ACR/ARHP Annual Meeting, 19–24 October 2018, Chicago, IL, USA.
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UK | September 2023 | 304956

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