ILARIS

Italy

Ilaris is used for the treatment of several inflammatory diseases, including periodic fever syndromes (such as CAPS, TRAPS, HIDS/MKD, and Familial Mediterranean Fever), Still's disease (including systemic juvenile idiopathic arthritis), and gouty arthritis.

Brand name ILARIS

Dosage form powder for solution for injection

Active substance / Dosage

canakinumab

Prescription type Restricted prescription – dispensable on hospital or specialist prescription

ATC code

L04AC08

Registration number 039472

Manufacturer NOVARTIS EUROPHARM LIMITED

ILARIS powder for solution for injection

Frequently asked questions

How should Ilaris be taken?

The medicinal product must be injected subcutaneously (into the tissue just under the skin). The method and frequency of injections (for example, every 4 or 8 weeks) depend on the specific disease being treated and must be determined by a doctor.

Who can inject Ilaris themselves?

Patients with periodic fever syndromes or Still's disease, who are at least 12 years old and weigh more than 40 kg, can self-inject the drug after receiving adequate training on the correct technique.

When should Ilaris not be used?

Do not use the medicinal product if you are allergic to the active substance or to any of the other components, or if you have or suspect you have a serious, active infection.

What are the common side effects of Ilaris?

Among the most common side effects, infections of various types (respiratory, ear, skin, or urinary tract), upper abdominal pain, joint pain, injection site reactions, and a decrease in white blood cells may be found.

What are the serious side effects that should be reported immediately?

It is necessary to contact a doctor immediately in case of fever lasting more than 3 days, signs of serious infection, allergic reactions (such as rash, itching, difficulty breathing, or palpitations), or if a widespread and atypical rash is noticed.

Are there interactions with other medicines or vaccines?

The use of tumor necrosis factor (TNF) inhibitor medicines together with Ilaris should be avoided. Furthermore, it is not recommended to take live vaccines during treatment; if necessary, the vaccine must be administered following specific timing indicated by the doctor.

How should the medicinal product be stored?

Ilaris must be stored in a refrigerator between 2°C and 8°C, protected from light in its original packaging. It must not be frozen.

Instructions for use

Table of contents

Ilaris 150 mg/ml solution for injection
1. What Ilaris is and what it is used for
2. What you should know before using Ilaris
3. How to use Ilaris
4. Possible side effects
5. How to store Ilaris
6. Package contents and other information
Instructions for the use of Ilaris solution for injection
Package leaflet: Information for the user
Ilaris 150 mg solution for injection in pre-filled pen
1. What Ilaris is and what it is used for
2. What you should know before using Ilaris
3. How to use Ilaris
4. Possible side effects
5. How to store Ilaris
6. Package contents and other information
Instructions for the use of Ilaris 150 mg Sensoready Pen
1. Important safety checks before
3. Cleaning the injection site:
4. Removing the cap:
5. Pen Position:
6. Start the injection:
7. Completing the injection:
8. Check that the green indicator fills the
9. Disposal of Ilaris 150 mg pre-filled pen:
Package leaflet: Information for the user
Ilaris 150 mg powder for injectable solution
1. What Ilaris is and what it is used for
2. What you need to know before using Ilaris
3. How to use Ilaris
4. Possible side effects
5. How to store Ilaris
6. Package contents and other information
Instructions for use of Ilaris powder for injectable solution

Package leaflet: Information for the user

Ilaris 150 mg/ml solution for injection

canakinumab
Please read all of this leaflet carefully before you start using this medicine, as it contains important information for you.

Keep this leaflet. You may need to read it again.
If you have any questions, ask your doctor, pharmacist or nurse.
This medicine has been prescribed for you only. Do not give it to other people, even if they have the same symptoms as yours, as it may be harmful.
If you experience any side effect, including those not listed in this leaflet, tell your doctor, pharmacist or nurse. See section 4. In addition to this package leaflet, you will be given a patient card containing important safety information you will need before and during treatment with Ilaris.

Contents of this leaflet:

What Ilaris is and what it is used for
What you need to know before using Ilaris
How to use Ilaris
Possible side effects
How to store Ilaris
Contents of the pack and other information

1. What Ilaris is and what it is used for

What Ilaris is
Ilaris contains the active substance canakinumab, a monoclonal antibody belonging to a group of medicines known as interleukin inhibitors. It blocks the activity in the body of a substance called interleukin-1 beta (IL-1 beta), which is present at higher levels in inflammatory diseases.

What Ilaris is used for
Ilaris is used to treat the following inflammatory diseases:

Periodic fever syndromes:
- Cryopyrin-Associated Periodic Syndromes (CAPS),
- Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS),
- Hyperimmunoglobulinemia D Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD),
- Familial Mediterranean Fever (FMF).
Still’s disease, including Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA)
Gouty arthritis

More information on each of these diseases is provided below.

Periodic fever syndromes
Ilaris is used in adults and children from 2 years of age for the treatment of:

Cryopyrin-Associated Periodic Syndromes (CAPS) – this is a group of autoinflammatory diseases including:
- Muckle-Wells Syndrome (MWS),
- Neonatal-Onset Multisystem Inflammatory Disease (NOMID), also called Chronic Infantile Neurological, Cutaneous, Articular Syndrome (CINCA),
- Severe forms of Familial Cold Autoinflammatory Syndrome (FCAS) / Familial Cold Urticaria (FCU) presenting with signs and symptoms extending beyond cold-induced urticarial rash.
Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)
Hyperimmunoglobulinemia D Syndrome (HIDS), also known as Mevalonate Kinase Deficiency (MKD)
Familial Mediterranean Fever (FMF): Ilaris is used for the treatment of FMF. Ilaris may be used in combination with colchicine, if appropriate.

In patients with periodic fever syndromes (CAPS, TRAPS, HIDS/MKD and FMF), the body produces too much IL-1 beta. This may cause fever, headache, fatigue, rash, or joint and muscle pain. By blocking the activity of IL-1 beta, Ilaris can improve these symptoms.

Still’s disease
Ilaris is used in adults, adolescents, and children for the treatment of Still’s disease, including Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA), in patients from 2 years of age, when other treatments have not worked well enough. Ilaris may be used alone or in combination with methotrexate.

Still’s disease, including SJIA and AOSD, is an inflammatory disease that can cause pain, swelling, and inflammation of one or more joints, as well as rash and fever. A pro-inflammatory protein called IL-1 beta plays an important role in the inflammation of Still’s disease. By blocking the activity of IL-1 beta, Ilaris can improve the signs and symptoms of Still’s disease.

Gouty arthritis
Ilaris is used in adults for the treatment of symptoms of frequent gouty arthritis attacks when other treatments have been insufficient.

Gouty arthritis is caused by the formation of urate crystals. These crystals trigger excessive production of IL-1 beta, which in turn can lead to sudden, severe joint pain, redness, warmth, and swelling (known as a gout attack). By blocking the activity of IL-1 beta, Ilaris can lead to improvement of these symptoms.

2. What you should know before using Ilaris

Do not use Ilaris

if you are allergic to canakinumab or to any of the other ingredients of this medicine (listed in section 6).
if you have or suspect you have an active, serious infection.

Warnings and precautions
Talk to your doctor before using Ilaris if any of the following apply to you:

if you currently have an infection or have had recurrent infections, or if you have a condition such as confirmed low white blood cell count, which may increase your risk of developing infections.
if you have or have had tuberculosis or have had close contact with someone who has active tuberculosis. Your doctor may perform a specific test to check whether you have tuberculosis.
if you have signs of liver problems such as yellowing of the skin or eyes, nausea, loss of appetite, dark-coloured urine, or pale-coloured stools.
if you need any vaccination. You are advised to avoid being vaccinated with a type of vaccine known as a live vaccine while being treated with Ilaris (see also “Other medicines and Ilaris”).
This medicine contains 0.4 mg of polysorbate 80 in each 1 ml of injectable solution. Polysorbates may cause allergic reactions. Inform your doctor if you or your child have known allergies.

Contact your doctor immediately

if you have previously developed widespread skin rash or extensive skin peeling after taking Ilaris. This severe skin reaction, known as DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms), has been rarely reported in association with Ilaris treatment, mainly in patients with systemic juvenile idiopathic arthritis (SJIA). Contact your doctor immediately if you notice an unusual and widespread skin rash, which may be accompanied by high fever and swollen lymph nodes.

Still’s disease

Patients with Still’s disease may develop a condition called macrophage activation syndrome (MAS), which can be life-threatening. Your doctor will monitor you for potential triggers of MAS, including infections and reactivation of underlying Still’s disease (flare-up).

Traceability
Each time you/your child receive a new package of Ilaris, it is important to record the name of the
medicine and the date of administration, together with the batch number, and to keep this information
in a safe place.

Children and adolescents

CAPS, TRAPS, HIDS/MKD, FMF and SJIA: Ilaris may be used in children from 2 years of age.
Gouty arthritis: Ilaris is not recommended for children or adolescents under 18 years of age.

Other medicines and Ilaris
Inform your doctor, pharmacist, or nurse if you are taking, have recently taken, or might take any other
medicines.

Live vaccines: You are advised to avoid vaccination with a type of vaccine known as a live vaccine while being treated with Ilaris. Your doctor may want to check your vaccination history and administer any missing vaccines before starting treatment with Ilaris. If you need vaccination with a live vaccine after starting treatment with Ilaris, discuss this with your doctor. A live vaccine should normally be given at least 3 months after your last dose of Ilaris and at least 3 months before your next dose.
Medicines called tumour necrosis factor (TNF) inhibitors, such as etanercept, adalimumab, or infliximab. These are mainly used for rheumatic and autoimmune diseases. They must not be used together with Ilaris, as they may increase the risk of infections.

Pregnancy and breastfeeding
If you are pregnant, think you may be pregnant, plan to become pregnant, or are breastfeeding, please
consult your doctor or pharmacist before taking this medicine.

You are advised to avoid becoming pregnant and to use adequate contraception while taking Ilaris and for at least 3 months after your last dose of Ilaris. It is important to inform your doctor if you are pregnant, think you may be pregnant, or are planning a pregnancy. Your doctor will discuss with you the potential risks of taking Ilaris during pregnancy.
If you were treated with canakinumab during pregnancy, it is important that you inform your child’s paediatrician or nurse before any vaccination is given to your child. Your child must not receive live vaccines until at least 16 weeks after you received your last dose of canakinumab before delivery.
It is not known whether Ilaris passes into breast milk. Your doctor will discuss with you the potential risks of treatment with Ilaris before breastfeeding.

Driving and using machines
Treatment with Ilaris may cause dizziness or marked tiredness (asthenia). You should bear this in mind when assessing your ability to perform tasks requiring judgment or motor skills. If you experience dizziness or tiredness, do not drive or operate tools or machinery until you feel well again.

3. How to use Ilaris

Use this medicine exactly as instructed by your doctor. If you have any doubts, consult your
doctor, pharmacist, or nurse.
Keep your doctor informed about your condition and any symptoms before using or before being
administered Ilaris (see section 2). Your doctor may decide to delay or interrupt treatment if necessary.
Ilaris is intended for subcutaneous use. This means it is injected through a short needle into the fatty
tissue just beneath the skin.
If you suffer from gouty arthritis, treatment must be supervised by a specialist doctor. Ilaris must be
injected only by healthcare professionals.
If you suffer from CAPS, TRAPS, HIDS/MKD, FMF, or Still’s disease (AOSD or SJIA), you may
self-inject Ilaris after adequate training, or someone assisting you may inject it for you.

How much Ilaris to use
Cryopyrin-associated periodic syndromes (CAPS)
The recommended initial dose of Ilaris is based on body weight:

Adults, adolescents, and children aged 4 years and older
- 150 mg for patients weighing more than 40 kg
- 2 mg/kg for patients weighing between 15 kg and 40 kg
- 4 mg/kg for patients weighing between 7.5 kg and less than 15 kg
Children aged 2 or 3 years
- 4 mg/kg for patients weighing 7.5 kg or more

Ilaris is injected as a single dose every 8 weeks.

If you have not had a satisfactory response to treatment after 7 days, your doctor may administer an additional dose of 150 mg or 2 mg/kg.
If you respond satisfactorily to this second dose, treatment will continue with 300 mg or 4 mg/kg every 8 weeks.
If you do not respond satisfactorily to the second dose, a third dose of Ilaris of 300 mg or 4 mg/kg may be administered.
If you respond satisfactorily to the third dose, treatment will continue with 600 mg or 8 mg/kg every 8 weeks.

For children treated with an initial dose of 4 mg/kg who have not had a satisfactory response after 7 days, your doctor may administer a second dose of 4 mg/kg. If the child responds satisfactorily to this dose, treatment may continue with a dose of 8 mg/kg every 8 weeks.

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS), hyperimmunoglobulinemia D syndrome (HIDS)/mevalonate kinase deficiency (MKD), and Familial Mediterranean Fever (FMF)
The recommended initial dose of Ilaris is based on body weight:

Adults and children aged 2 years and older
- 150 mg for patients weighing more than 40 kg
- 2 mg/kg for patients weighing between 7.5 kg and less than 40 kg

Ilaris is injected as a single dose every 4 weeks.

If you have not responded satisfactorily to treatment after 7 days, your doctor may administer another dose of 150 mg or 2 mg/kg.
If you respond satisfactorily to this dose, treatment will continue with 300 mg or 4 mg/kg every 4 weeks.

Still’s disease (SJIA and AOSD)
The recommended dose of Ilaris for patients with Still’s disease and body weight of 7.5 kg or more is 4 mg/kg (up to a maximum of 300 mg). Ilaris is injected as a single dose every 4 weeks.

Gouty arthritis
Your doctor will discuss with you the need to initiate or adjust uric acid-lowering therapy (uricosuric therapy) to reduce uric acid levels in the blood.
The recommended dose of Ilaris for adult patients with gouty arthritis is 150 mg, administered as a single dose at the onset of a gouty arthritis attack.
If further treatment with Ilaris is needed and you have experienced relief from the last dose, you must wait at least 12 weeks before receiving the next dose.

Self-injection of Ilaris or how to inject Ilaris to a patient
If you are a patient with CAPS, TRAPS, HIDS/MKD, FMF, or Still’s disease (AOSD or SJIA), or if you are assisting a patient with one of these conditions, you may self-inject Ilaris after appropriate training in the correct injection technique.

The patient or caregiver and the doctor must decide together who will administer the Ilaris injections.
Your doctor or nurse will show you how to administer the Ilaris injection.
If you have not been properly trained or if you are unsure how to proceed, do not attempt to self-inject.
Ilaris 150 mg/ml solution for injection is supplied in a single-use vial for personal use.
Never reuse any leftover solution.

For instructions on how to inject Ilaris, read the section “Instructions for use” at the end of this
package leaflet. If you have any doubts, consult your doctor, pharmacist, or nurse.

How long to use Ilaris

CAPS, TRAPS, HIDS/MKD, FMF, or Still’s disease (AOSD or SJIA): You must continue using Ilaris for the duration specified by your doctor.
Gouty arthritis: If you experience a gouty arthritis attack, you will be administered a single dose of Ilaris. If you experience a new attack, your doctor may consider administering another dose, but not earlier than 12 weeks after the previous dose.

If you use more Ilaris than you should
If you accidentally inject more Ilaris than the recommended dose, it is unlikely to be serious, but you must inform your doctor, pharmacist, or nurse as soon as possible.

If you forget to use Ilaris
If you have CAPS, TRAPS, HIDS/MKD, FMF, or Still’s disease (AOSD or SJIA) and have forgotten to inject a dose of Ilaris, inject the missed dose as soon as you remember. Then inform your doctor to discuss when you should administer the next dose. Continue with injections at the recommended intervals as before.

If you stop using Ilaris
Stopping treatment with Ilaris may cause your condition to worsen. Do not stop treatment with Ilaris unless instructed to do so by your doctor.
If you have any questions about using this medicine, consult your doctor, pharmacist, or nurse.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody will get them.
Some side effects can be serious. If you notice any of the following side effects, tell your doctor
immediately:

Fever lasting more than 3 days or any other symptom that may indicate a serious infection. These include shaking, chills, feeling unwell, loss of appetite, generalised aches, usually associated with sudden onset of illness, sore throat or mouth ulcers, cough, mucus, chest pain, difficulty breathing, ear pain, prolonged headache or localised redness, warmth or swelling of the skin or inflammation of connective tissue (cellulitis). These symptoms may be caused by a serious infection, an unusual infection (opportunistic infection), or may be related to low levels of white blood cells (known as leucopenia or neutropenia). If necessary, your doctor may perform regular blood tests.
Allergic reactions with skin rash and itching, and possibly also hives, difficulty breathing or swallowing, dizziness, unusual awareness of heartbeat (palpitations), or low blood pressure.

Other side effects of Ilaris include:
Very common (may affect more than 1 in 10 people):

Infections of any type. These may include:
- Respiratory infections such as chest infection, flu, sore throat, runny nose, stuffy nose, sneezing, feeling of pressure or pain in the cheeks or forehead with or without fever (pneumonia, bronchitis, influenza, sinusitis, rhinitis, pharyngitis, tonsillitis, nasopharyngitis, upper respiratory tract infection).
- Other infections such as ear infection, skin infection (cellulitis), stomach pain and feeling unwell (gastroenteritis), and painful or frequent urination with or without fever (urinary tract infection).
Upper abdominal pain.
Joint pain (arthralgia).
Decrease in white blood cell count (leucopenia).
Abnormal kidney function test results (decreased creatinine clearance, proteinuria).
Injection site reaction (such as redness, swelling, warmth and itching).

Common (may affect up to 1 in 10 people):

Candida – yeast infection of the vagina (vulvovaginal candidiasis)
Feeling dizzy, sensation of spinning (dizziness or vertigo).
Back or muscle pain.
Feeling weak or very tired (fatigue, asthenia).
Decrease in levels of white blood cells that help prevent infection (neutropenia).
Abnormal blood levels of triglycerides (lipid metabolism disorder).
Abnormal liver function tests (increased transaminases) or high levels of bilirubin in the blood with or without yellowing of the skin and eyes (hyperbilirubinemia).

Uncommon (may affect up to 1 in 100 people):

Heartburn (gastro-oesophageal reflux disease).
Decrease in levels of blood cells that help prevent bleeding (platelets).

Tell your doctor or paediatrician immediately if you notice any of these symptoms.
Reporting of side effects
If you experience any side effect, including those not listed in this leaflet, talk to your doctor, pharmacist or nurse. You can also report side effects directly through the national reporting system listed in Annex V. By reporting side effects, you can help provide more information on the safety of this medicine.

5. How to store Ilaris

Keep this medicine out of the sight and reach of children.
Do not use this medicine after the expiry date which is stated on the label after “EXP” and on the carton after “Scad.”. The expiry date refers to the last day of that month.
Store in a refrigerator (2°C - 8°C). Do not freeze.
Keep the vial in its carton to protect the medicine from light.
The solution should be used immediately after the first puncture of the vial stopper for injection preparation.
Do not use this medicine if you notice that the solution is not clear or opalescent or contains particles.
Any unused medicine must be discarded after the dose has been injected.
Do not dispose of any medicine via wastewater or household waste. Ask your pharmacist how to dispose of medicines you no longer use. This will help protect the environment.

6. Package contents and other information

What Ilaris contains

The active substance is canakinumab. Each vial contains 150 mg of canakinumab in 1 ml of solution.
The other components are mannitol, histidine, monohydrochloride monohydrate of histidine, polysorbate 80 (see section 2), water for injections.

Description of the appearance of Ilaris and contents of the pack

Ilaris is supplied as an injectable solution in a 2 ml glass vial.
The solution is a liquid ranging from transparent to opalescent, colourless to slightly brownish yellow. Do not use if the liquid contains readily visible particles, is cloudy, or distinctly brown in colour.
Ilaris is available in packs containing one vial.

Marketing Authorisation Holder
Novartis Europharm Limited
Vista Building
Elm Park, Merrion Road
Dublin 4
Ireland
Manufacturer
Novartis Farmacéutica, S.A.
Gran Vía de les Corts Catalanes, 764
08013 Barcelona
Spain
Novartis Pharma GmbH
Roonstrasse 25
90429 Nuremberg
Germany
Lek Pharmaceuticals d.d.
Verovskova Ulica 57
1526 Ljubljana
Slovenia
Novartis Pharma GmbH
Sophie-Germain-Strasse 10
90443 Nuremberg
Germany
For further information about this medicinal product, please contact the local representative of the Marketing Authorisation Holder:
België/Belgique/Belgien Lietuva
Novartis Pharma N.V. SIA Novartis Baltics Lietuvos filialas
Tél/Tel: +32 2 246 16 11 Tel: +370 5 269 16 50
България Luxembourg/Luxemburg
Novartis Bulgaria EOOD Novartis Pharma N.V.
Тел.: +359 2 489 98 28 Tél/Tel: +32 2 246 16 11
Česká republika Magyarország
Novartis s.r.o. Novartis Hungária Kft.
Tel: +420 225 775 111 Tel.: +36 1 457 65 00
Danmark Malta
Novartis Healthcare A/S Novartis Pharma Services Inc.
Tlf.: +45 39 16 84 00 Tel: +356 2122 2872
Deutschland Nederland
Novartis Pharma GmbH Novartis Pharma B.V.
Tel: +49 911 273 0 Tel: +31 88 04 52 111
Eesti Norge
SIA Novartis Baltics Eesti filiaal Novartis Norge AS
Tel: +372 66 30 810 Tlf: +47 23 05 20 00
Ελλάδα Österreich
Novartis (Hellas) A.E.B.E. Novartis Pharma GmbH
Τηλ: +30 210 281 17 12 Tel: +43 1 86 6570
España Polska
Novartis Farmacéutica, S.A. Novartis Poland Sp. z o.o.
Tel: +34 93 306 42 00 Tel.: +48 22 375 4888
France Portugal
Novartis Pharma S.A.S. Novartis Farma - Produtos Farmacêuticos, S.A.
Tél: +33 1 55 47 66 00 Tel: +351 21 000 8600
Hrvatska România
Novartis Hrvatska d.o.o. Novartis Pharma Services Romania SRL
Tel. +385 1 6274 220 Tel: +40 21 31299 01
Ireland Slovenija
Novartis Ireland Limited Novartis Pharma Services Inc.
Tel: +353 1 260 12 55 Tel: +386 1 300 75 50
Ísland Slovenská republika
Vistor hf. Novartis Slovakia s.r.o.
Sími: +354 535 7000 Tel: +421 2 5542 5439
Italia Suomi/Finland
Novartis Farma S.p.A. Novartis Finland Oy
Tel: +39 02 96 54 1 Puh/Tel: +358 (0)10 6133 200
Κύπρος Sverige
Novartis Pharma Services Inc. Novartis Sverige AB
Τηλ: +357 22 690 690 Tel: +46 8 732 32 00
Latvija
SIA Novartis Baltics
Tel: +371 67 887 070
Other sources of information
More detailed information on this medicinal product is available on the European Medicines Agency website: https://www.ema.europa.eu/

Instructions for the use of Ilaris solution for injection

Read these instructions every time before injection.

It is important that you do not attempt to self-inject the medicine until you have been trained by a healthcare professional.
See also section 3, "Self-injection of Ilaris or injection of Ilaris to a patient".

Essential preparations

Identify a clean area where you can prepare and self-administer the injection.
Wash your hands thoroughly with soap and water, then dry them with a clean towel.
After removing the vial from the refrigerator, check the expiration date on the vial. Do not use after the expiration date stated on the label and carton. The expiration date refers to the last day of the month.
Allow the unopened vial to sit for 10 minutes to bring the contents to room temperature. Do not attempt to warm the vial. Let it warm up on its own.
Always use new, unopened needles and syringes. Do not touch the needles or the top of the vial.

Preparation of required materials
Included in the package

one vial of Ilaris solution for injection (store in refrigerator)

Not included in the package

one 1.0 ml syringe
one needle (e.g. 18 G or 21 G x 2 inch, or similar, as available on the market) for drawing the solution from the vial ("withdrawal needle")
one needle 27 G x 0.5 inch (or similar, as available on the market) for injection ("injection needle")
alcohol swabs
clean, dry cotton pads
one adhesive bandage
a dedicated container for the disposal of used needles, syringe, and vial (sharps container)

Preparation of the injection

Two hands preparing the medication, one holding a vial and the

1. Remove the protective cap from the Ilaris vial. Do not touch the rubber stopper of the vial. Clean the rubber stopper with an alcohol swab. Open the syringe and the transfer needle packaging. − Attach the transfer needle to the syringe. − Remove the protective cap from the transfer needle. − Insert the transfer needle into the Ilaris vial through the center of the rubber stopper.

Illustration of a graduated syringe with needle inserted into the

2. Gently tap the vial to ensure that the required amount of solution can be withdrawn into the syringe. NOTE: The required volume depends on the dose to be administered. You will be instructed by healthcare personnel on the correct volume for you. 3. Gently pull back the syringe plunger to the correct mark (volume to withdraw as instructed by healthcare provider), filling the syringe with Ilaris solution. If you see air bubbles in the syringe, remove them as instructed by healthcare personnel. Make sure the syringe contains the correct volume of solution. 4. Remove the syringe and transfer needle from the vial. (There may be residual solution left in the vial.) Cover the transfer needle with its protective cap as instructed by healthcare or pharmacy staff. Remove the transfer needle from the syringe and dispose of it in a sharps container. 5. Open the package containing the injection needle and attach the needle to the syringe. Administer the injection immediately.

Administer the injection

Black and white drawing showing a hand holding a syringe to inject medication into the upper thigh of a seated person

6. Choose an injection site on the upper thigh, abdomen, upper arm, or buttocks. Do not use an area with a rash or lesions, or that is bruised or not smooth. Do not inject into scar tissue, as this may result in incomplete absorption of the medication. Avoid injecting into a vein. 7. Clean the injection site with a new alcohol swab. Allow the area to dry. Remove the needle cap prior to injection. 8. Gently pinch the skin at the injection site. Hold the syringe at a 90-degree angle and, in one quick motion, insert the needle completely into the skin.

Schematic drawing of a person holding a syringe in the right hand to inject medication into the left upper thigh

9. Keep the needle fully inserted into the skin while slowly pushing the syringe plunger until the reservoir is empty. Release the pinched skin and immediately withdraw the needle. Dispose of the needle and syringe in a sharps container without recapping or removing the needle.

After injection

Stylized drawing of a hand pressing a square patch onto the upper thigh of a seated person

10. Do not rub the injection site. If bleeding occurs, apply a clean, dry cotton pad to the area and press gently for 1 or 2 minutes or until bleeding stops. Then apply a plaster.

A hand holding a syringe with needle, inserting it into a sharps disposal container for sharp and hazardous objects

11. Dispose of needles and syringes safely in a sharps container or as instructed by healthcare professionals or the pharmacist. Syringes and needles must never be reused. 12. Properly discard vials containing Ilaris solution (if present) as instructed by healthcare professionals or the pharmacist. Unused medicine or waste material derived from this medicine must be disposed of in accordance with local regulations. Never reuse any leftover solution. Keep sharps containers out of sight and reach of children. Dispose of them as instructed by healthcare professionals or the pharmacist.

Package leaflet: Information for the user

Ilaris 150 mg solution for injection in pre-filled pen

canakinumab
Please read this leaflet carefully before using this medicine because it contains important information for you.

Keep this leaflet. You may need to read it again.
If you have any questions, ask your doctor, pharmacist, or nurse.
This medicine has been prescribed for you only. Do not give it to others, even if their symptoms are the same as yours, as it may be harmful.
If you experience any adverse reactions, including those not listed in this leaflet, contact your doctor, pharmacist, or nurse. See section 4. In addition to this package leaflet, you will be provided with a patient alert card containing important safety information you will need before and during treatment with Ilaris.

Contents of this leaflet:

What Ilaris is and what it is used for
What you need to know before using Ilaris
How to use Ilaris
Possible side effects
How to store Ilaris
Contents of the pack and other information

1. What Ilaris is and what it is used for

What Ilaris is used for
Ilaris is used to treat the following inflammatory diseases:

Periodic fever syndromes:
- Cryopyrin-Associated Periodic Syndromes (CAPS),
- Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS),
- Hyperimmunoglobulinemia D syndrome (HIDS)/mevalonate kinase deficiency (MKD),
- Familial Mediterranean Fever (FMF).
Still’s disease, including Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA)
Gouty arthritis

More information on each of these diseases is provided below.

Periodic fever syndromes
Ilaris is used in adults and children from 2 years of age for the treatment of:

Cryopyrin-Associated Periodic Syndromes (CAPS) – this is a group of autoinflammatory diseases including:
- Muckle-Wells Syndrome (MWS),
- Neonatal-Onset Multisystem Inflammatory Disease (NOMID), also known as Chronic Infantile Neurological, Cutaneous and Articular Syndrome (CINCA),
- Severe forms of Familial Cold Autoinflammatory Syndrome (FCAS)/Familial Cold Urticaria (FCU) presenting with signs and symptoms extending beyond cold-induced urticarial rash.
Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)
Hyperimmunoglobulinemia D syndrome (HIDS), also known as mevalonate kinase deficiency (MKD)
Familial Mediterranean Fever (FMF): Ilaris is used for the treatment of FMF. Ilaris may be used in combination with colchicine, if appropriate.

In patients with periodic fever syndromes (CAPS, TRAPS, HIDS/MKD and FMF), the body produces too much IL-1 beta. This can cause fever, headache, fatigue, rash, or joint and muscle pain. By blocking the activity of IL-1 beta, Ilaris can improve these symptoms.

Still’s disease
Ilaris is used in adults, adolescents, and children for the treatment of Still’s disease, including Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA), in patients from 2 years of age, when other treatments have not worked well enough. Ilaris can be used alone or in combination with methotrexate.

Still’s disease, including SJIA and AOSD, is an inflammatory disease that can cause pain, swelling, and inflammation of one or more joints, as well as rash and fever. A pro-inflammatory protein called IL-1 beta plays an important role in the inflammation associated with Still’s disease. By blocking the activity of IL-1 beta, Ilaris can improve the signs and symptoms of Still’s disease.

Gouty arthritis
Ilaris is used in adults for the treatment of symptoms of frequent gouty arthritis attacks when other treatments have been insufficient.

2. What you should know before using Ilaris

Do not use Ilaris

if you are allergic to canakinumab or to any of the other ingredients of this medicine (listed in section 6).
if you have or suspect you have an active, serious infection.

Warnings and precautions
Talk to your doctor before using Ilaris if any of the following apply to you:

if you currently have an infection, or have had recurrent infections, or have a condition such as a confirmed low white blood cell count, which may increase your risk of developing infections.
if you have or have had tuberculosis or have had close contact with someone who has active tuberculosis. Your doctor may perform a specific test to check whether you have tuberculosis.
if you have signs of liver problems such as yellowing of the skin or eyes, nausea, loss of appetite, dark-coloured urine, or pale-coloured stools.
if you need any vaccination. You are advised to avoid being vaccinated with a type of vaccine known as a live vaccine while being treated with Ilaris (see also “Other medicines and Ilaris”).
This medicine contains 0.4 mg of polysorbate 80 in each 1 ml of injectable solution. Polysorbates may cause allergic reactions. Inform your doctor if you or your child have known allergies.

Contact your doctor immediately

if you have previously developed widespread skin rash or extensive skin peeling after taking Ilaris. This severe skin reaction, known as DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms), has been rarely reported in association with Ilaris treatment, particularly in patients with systemic juvenile idiopathic arthritis (SJIA). Contact your doctor immediately if you notice an unusual and widespread skin rash, which may be accompanied by high fever and swollen lymph nodes.

Still’s disease

Patients with Still’s disease may develop a condition called macrophage activation syndrome (MAS), which can be life-threatening. Your doctor will monitor you for potential triggers of MAS, including infections and reactivation of underlying Still’s disease (flare-up).

Traceability
Each time you/your child receive a new package of Ilaris, it is important to record the name of the medicine and the date of administration, together with the batch number, and to keep this information in a safe place.

Children and adolescents

CAPS, TRAPS, HIDS/MKD, FMF and SJIA: Ilaris may be used in children from 2 years of age.
Gouty arthritis: Ilaris is not recommended for children or adolescents under 18 years of age.

Other medicines and Ilaris
Inform your doctor, pharmacist, or nurse if you are taking, have recently taken, or might take any other medicines.

Live vaccines: You are advised to avoid vaccination with a type of vaccine known as a live vaccine while being treated with Ilaris. Your doctor may check your vaccination history and administer any missing vaccines before starting treatment with Ilaris. If you need vaccination with a live vaccine after starting Ilaris treatment, discuss this with your doctor. A live vaccine should normally be administered at least 3 months after your last dose of Ilaris and at least 3 months before your next dose.
Medicines called tumour necrosis factor (TNF) inhibitors, such as etanercept, adalimumab, or infliximab. These are mainly used for rheumatic and autoimmune diseases. They must not be used together with Ilaris, as they may increase the risk of infections.

Pregnancy and breastfeeding
If you are pregnant, think you may be pregnant, planning to become pregnant, or are breastfeeding, ask your doctor or pharmacist for advice before taking this medicine.

You are advised to avoid becoming pregnant and to use effective contraception while taking Ilaris and for at least 3 months after your last dose. It is important to inform your doctor if you are pregnant, think you may be pregnant, or are planning a pregnancy. Your doctor will discuss with you the potential risks of taking Ilaris during pregnancy.
If you were treated with canakinumab during pregnancy, it is important to inform your child’s paediatrician or nurse before any vaccination is given to your child. Your child must not receive live vaccines until at least 16 weeks after you received your last dose of canakinumab before delivery.
It is not known whether Ilaris passes into breast milk. Your doctor will discuss with you the potential risks of treatment with Ilaris before breastfeeding.

Driving and using machines
Treatment with Ilaris may cause dizziness or marked fatigue (asthenia). This should be taken into account when assessing your ability to perform tasks requiring judgment or motor skills. If you experience dizziness or fatigue, do not drive or operate tools or machinery until you feel well again.