Ilaris 150 mg powder for solution for injection

Ilaris is used in adults, adolescents and children aged 4 years and older with body weight above 15 kg to treat the following auto-inflammatory diseases, which are collectively known as Cryopyrin-Associated Periodic Syndromes (CAPS):
- 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 beyond cold-induced urticarial skin rash.

Ilaris belongs to a group of medicines called interleukin inhibitors. The active substance in Ilaris is canakinumab, a fully-human monoclonal antibody. It blocks the activity of a substance called interleukin-1 beta (IL-1 beta). In patients with CAPS, the body produces excessive amounts of IL-1 beta. This may lead to symptoms such as fever, headache, fatigue, skin rash, or painful joints and muscles. By blocking the activity of IL-1 beta, canakinumab leads to an improvement in these symptoms.

If you have any questions about how Ilaris works or why this medicine has been prescribed for you, ask your doctor.

Do not use Ilaris

• if you are allergic (hypersensitive) to canakinumab or any of the other ingredients of Ilaris listed in section 6 of this leaflet.
• if you have an active, severe infection. If you think you may be allergic or have an infection, do not use Ilaris and ask your doctor for advice.

Take special care with Ilaris
Before using Ilaris, tell your doctor if any of the following applies to you:
- if you currently have an infection or if you have a history of recurring infections or a condition which makes you vulnerable to infections.
- if you require vaccinations. You are advised to avoid a certain type of vaccination (also called live vaccines) while being treated with Ilaris (see also ?Using other medicines?). During treatment with Ilaris, tell your doctor immediately if you experience any of the following symptoms:
- prolonged fever (i.e. fever lasting longer than 3 days) or any other symptoms possibly related to an infection, such as prolonged cough, prolonged headache or localised redness, warmth or swelling of your skin.- signs of an allergic reaction such as difficulty breathing, nausea, dizziness, skin rash, palpitations or low blood pressure.
- signs of liver disorders such as yellow skin and eyes, nausea, loss of appetite, dark-coloured urine and light-coloured stools.
Ilaris is not recommended for children younger than 4 years of age or who weigh less than 15 kg.

Using other medicines

Please tell your doctor, nurse or pharmacist if you are taking or have recently taken any other medicines, including medicines obtained without a prescription.
- You are advised to avoid a certain type of vaccination known as ?live vaccines? while being treated with Ilaris. Your doctor may want to check your vaccination history and give you any vaccinations that you have missed before you start treatment with Ilaris. Should vaccination with live vaccines be necessary after initiation of Ilaris treatment, you are recommended to wait for at least 3 months after the last Ilaris injection and before the next one.
- Medicines called tumour necrosis factor (TNF) inhibitors predominantly used in rheumatoid and autoimmune diseases (such as etanercept, adalimumab or infliximab) should not be used with Ilaris because this may increase the risk of infections.

Pregnancy and breast-feeding

- Ilaris has not been studied in pregnant women. You are advised to avoid becoming pregnant and must use adequate contraception while using Ilaris and for at least 3 months after the last Ilaris treatment. It is important to tell your doctor if you are pregnant, if you think you may be pregnant or if you plan to get pregnant. Your doctor will discuss with you the potential risk of taking Ilaris during pregnancy.
- It is not known whether Ilaris passes into human milk. Your doctor will discuss with you the potential risks of taking Ilaris before breast-feeding.

Driving and using machines

Some symptoms associated with CAPS or with Ilaris treatment, such as a spinning sensation (known as vertigo), may affect your ability to drive or use machines. If you feel a spinning sensation, do not drive or operate any tools or machines until you are feeling normal again.

Ask your doctor, nurse or pharmacist for advice before taking any medicine.

Always use Ilaris exactly as your doctor has told you. You should check with your doctor, nurse or pharmacist if you are not sure.

Ilaris is intended for subcutaneous use. This means that it is injected through a short needle into the fatty tissue just under the skin.

How much Ilaris to use

The recommended dose of Ilaris for CAPS patients is:
- 150 mg for patients with body weight of more than 40 kg.
- 2 mg/kg for patients with body weight between 15 kg and 40 kg.
Ilaris is injected as a single dose every 8 weeks.

If you have not sufficiently responded to treatment after 7 days, your doctor may consider giving you a repeat dose of 150 mg or 2 mg/kg. After this you will continue to receive this higher dose of 300 mg or 4 mg/kg.

Injecting Ilaris yourself

After proper training in the correct injection technique, you may inject Ilaris yourself. - You and your doctor should decide together whether or not you will inject Ilaris yourself. - Your doctor or nurse will show you how to inject yourself.
- Do not try to inject yourself if you have not been properly trained or if you are not sure how to do it.
For instructions on how to inject yourself with Ilaris, please read the section ?Instructions for use? at the end of this leaflet. If you have any questions, contact your doctor, nurse or pharmacist.

How long to use Ilaris

You should continue using Ilaris for as long as your doctor tells you.

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 should inform your doctor, nurse or pharmacist as soon as possible. - You should not inject Ilaris earlier than 8 weeks after the previous dose, unless your doctor tells you to. If you accidentally inject Ilaris sooner than you should, you should also inform your doctor, nurse or pharmacist as soon as possible.

If you forget to use Ilaris

If you have forgotten to use a dose of Ilaris, inject the next dose as soon as you remember, then contact your doctor to discuss when you should take the next dose. You should then continue with injections at 8-week intervals as before.

If you have any further questions on the use of this medicine, ask your doctor, nurse or pharmacist.

Like all medicines, Ilaris may cause side effects, although not everybody gets them. Most of the side effects are mild to moderate and will generally disappear a few days to a few weeks after treatment.

Side effects may occur with certain frequencies, which are defined as follows:

Very common affects more than 1 user in 10 Common affects 1 to 10 users in 100 Uncommon affects 1 to 10 users in 1,000 Rare affects 1 to 10 users in 10,000 Very rare affects less than 1 user in 10,000 Not known frequency cannot be estimated from the available data.

Potentially serious side effects (very common):

- Prolonged fever (i.e. fever lasting longer than 3 days) or any other symptoms possibly related to an infection, such as prolonged cough, prolonged headache or localised redness, warmth or swelling of your skin.
If you experience any of these, tell your doctor immediately.

Very common side effects:

- Spinning sensation (vertigo).
- Injection site reaction (such as redness, swelling, warmth and itching).

Common side effects:

- Sore throat and nose (nasopharyngitis).

Potential side effects:

These side effects could potentially occur but have either not been reported as a side effect with Ilaris or have been reported rarely during the use of Ilaris but seem to be unrelated to Ilaris. - Signs of an allergic reaction such as difficulty breathing, nausea, dizziness, skin rash, palpitations or low blood pressure. If you experience any of these symptoms, tell your doctor immediately.
- Signs of liver disorders such as yellow skin and eyes, nausea, loss of appetite, dark-coloured urine and light-coloured stools.

If any of the side effects gets serious, or if you notice any side effects not listed in this leaflet, please tell your doctor, nurse or pharmacist.

Keep out of the reach and sight of children.

Do not use Ilaris after the expiry date which is stated on the label and carton. The expiry date refers to the last day of that month.

Store in a refrigerator (2°C - 8°C).
Do not freeze.
Store in the original package in order to protect from light.

After reconstitution, from a microbiological point of view, the product should be used immediately. However, chemical and physical in-use stability has been demonstrated for 24 hours at 2°C - 8°C.

Do not use Ilaris if you notice that the solution is not clear to opalescent or contains particles.

Medicines should not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of medicines no longer required. These measures will help to protect the environment.

What Ilaris contains

- The active substance is canakinumab. One vial of powder contains 150 mg canakinumab.- The other ingredients are:sucrose, histidine, histidine hydrochloride monohydrate, polysorbate 80.

What Ilaris looks like and contents of the pack

- Ilaris is supplied as a powder for solution for injection in a 150 mg glass vial. - Ilaris is available in packs containing one vial or multipacks comprising four intermediate packs, each containing one vial. Not all pack sizes may be marketed in your country. - The powder is white.

Marketing Authorisation Holder

Novartis Europharm Limited
Wimblehurst Road
Horsham
West Sussex, RH12 5AB
United Kingdom

Manufacturer

Novartis Pharma GmbH
Roonstrasse 25
D-90429 Nuremberg
Germany

For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder:

BelgiëBelgiqueBelgien Novartis Pharma N.V. TélTel 32 2 246 16 11 LuxembourgLuxemburg Novartis Pharma N.V. TélTel 32 2 246 16 11

Novartis Pharma Services Inc. . 359 2 489 98 28 Magyarország Novartis Hungária Kft. Pharma Tel. 36 1 457 65 00

eská republika Novartis s.r.o. Tel 420 225 775 111 Malta Novartis Pharma Services Inc. Tel 356 2298 3217

Danmark Novartis Healthcare AS Tlf 45 39 16 84 00 Nederland Novartis Pharma B.V. Tel 31 26 37 82 111

Deutschland Novartis Pharma GmbH Tel 49 911 273 0 Norge Novartis Norge AS Tlf 47 23 05 20 00

Eesti Novartis Pharma Services Inc. Tel 372 66 30 810 Österreich Novartis Pharma GmbH Tel 43 1 86 6570

Novartis Hellas A.E.B.E. 30 210 281 17 12 Polska Novartis Poland Sp. z o.o. Tel. 48 22 550 8888

España Novartis Farmacéutica, S.A. Tel 34 93 306 42 00 Portugal Novartis Farma - Produtos Farmacêuticos, S.A. Tel 351 21 000 8600

France Novartis Pharma S.A.S. Tél 33 1 55 47 66 00 România Novartis Pharma Services Inc. Tel 40 21 31299 01

Ireland Novartis Ireland Limited Tel 353 1 260 12 55 Slovenija Novartis Pharma Services Inc. Tel 386 1 300 75 50

Ísland Vistor hf. Sími 354 535 7000 Slovenská republika Novartis Slovakia s.r.o. Tel 421 2 5542 5439

Italia Novartis Farma S.p.A. Tel 39 02 96 54 1 SuomiFinland Novartis Finland Oy PuhTel 358 9 61 33 22 11

357 22 690 690 Sverige Novartis Sverige AB Tel 46 8 732 32 00

Latvija Novartis Pharma Services Inc. Tel 371 67 887 070 United Kingdom Novartis Pharmaceuticals UK Ltd. Tel 44 1276 698370

Lietuva
Novartis Pharma Services Inc.
Tel: +370 5 269 16 50

This leaflet was last approved in

This medicine has been authorised under ?Exceptional Circumstances?. This means that because of the rarity of this disease it has been impossible to get complete information on this medicine. The European Medicines Agency will review any new information on the medicine every year and this leaflet will be updated as necessary.

INSTRUCTIONS FOR USE OF ILARIS POWDER FOR SOLUTION FOR INJECTION

Please note that the preparation of the injection at room temperature takes about 30 minutes. See also section 3, ?Injecting Ilaris yourself?.

Read these instructions all the way through before beginning.

Essential preparation

- Find a clean place in which to prepare and administer the injection.
- Wash your hands with soap and water.
- Check the expiry dates on the vial and syringes. Do not use after the expiry date which is stated on the label and carton. The expiry date refers to the last day of that month. - Always use new, unopened needles and syringes. Avoid touching the needles and the tops of the vials.

Gather together the necessary items

Included in the pack
One vial of Ilaris powder for solution for injection (keep refrigerated)

Not included in the pack one vial or ampoule of sterile water for injection water do not refrigerate one 1.0 ml syringe one 18 G x 2 inch 50 mm needle for reconstituting the powder transfer needle one 27 G x 0.5 inch 13 mm needle for injecting injection needle alcohol swabs clean, dry cotton swabs an adhesive plaster a proper disposal container for used needles, syringe and vials sharps container

Mixing Ilaris

1. Remove the protective caps from the Ilaris and water vials. Do not touch the vial stoppers. Clean the stoppers with the alcohol swab. 2. Open the wrappers containing the syringe and the transfer needle bigger one and attach the needle to the syringe. 3. Carefully remove the cap from the transfer needle and set the cap aside. Pull the plunger all the way down to the 1.0 ml mark, filling the syringe with air. Insert the needle into the water vial through the centre of the rubber stopper Fig. 1. 4. Gently push the plunger all the way down until air is injected into the vial.

5. Invert the vial and syringe assembly and bring to eye level Fig. 2. 6. Make sure the tip of the transfer needle is covered by the water and slowly pull the syringe plunger down to slightly past the 1.0 ml mark. If you see bubbles in the syringe, remove bubbles as instructed by your healthcare provider or pharmacist. 7. Make sure 1.0 ml of water is in the syringe, then withdraw the needle from the vial. There will be water remaining in the vial. 8. Insert the transfer needle through the centre of the stopper of the vial of Ilaris powder, taking care not to touch the needle or the stopper. Slowly inject 1.0 ml of water in to the vial containing the Ilaris powder Fig. 3. 9. Carefully remove the syringe with the transfer needle from the vial and recap the needle as instructed by your healthcare provider or pharmacist. 10. Without touching the rubber stopper, swirl do not shake the vial slowly at an angle of about 45 degrees for approximately 1 minute Fig. 4a. Allow to stand for 5 minutes. 11. Now, gently turn the vial upside down and back again ten times, again taking care not to touch the rubber stopper Fig. 4b. 12. Allow to stand for about 15 minutes at room temperature to obtain a clear to opalescent solution. Do not shake. Do not use if particles are present in the solution. 13. Make sure all of the solution is in the bottom of the vial. If drops remain on the stopper, tap the side of the vial to remove them. The solution should be clear to opalescent and free of visible particles. If not used immediately after reconstitution, the solution should be stored in the refrigerator 2C to 8C and used within 24 hours.

Preparing the injection

14. Clean the rubber stopper of the vial containing the Ilaris solution with a new alcohol swab. 15. Uncap the transfer needle again. Pull the plunger of the syringe all the way down to the 1.0 ml mark, filling the syringe with air. Insert the syringe needle into the vial of Ilaris solution through the centre of the rubber stopper Fig. 5. Gently push the plunger all the way down until air is injected into the vial. Do not inject air into the medicine. 16. Do not invert the vial and syringe assembly Fig. 6a. Insert the needle all the way into the vial until it reaches the bottom edge. 17. Tip the vial to ensure that the required amount of solution can be drawn into the syringe Fig. 6b. NOTE The required amount depends on the dose to be administered 0.2 ml to 1.0 ml. Your healthcare provider will instruct you on the right amount for you. 18. Slowly pull the syringe plunger up to the correct mark 0.2 ml to 1.0 ml, filling the syringe with Ilaris solution. If there are air bubbles in the syringe, remove bubbles as instructed by your healthcare provider. Ensure that the correct amount of solution is in the syringe. 19. Remove the syringe and needle from the vial. There may be solution remaining in the vial. Recap the transfer needle as instructed by your healthcare provider or pharmacist. Remove the transfer needle from the syringe. Place the transfer needle in the sharps container. 20. Open the wrapper containing the injection needle and attach the needle to the syringe. Set the syringe aside.

Giving the injection

21. Choose an injection site on the upper thigh, abdomen, upper arm or buttocks. Do not use an area that has a rash or broken skin, or is bruised or lumpy. Avoid injecting into scar-tissue as this may lead to insufficient exposure to canakinumab. Avoid injecting in to a vein. 22. Clean the injection site with a new alcohol swab. Allow the area to dry. Uncap the injection needle. 23. Gently pinch the skin up at the injection site. Hold the syringe at a 90-degree angle and in a single, smooth motion, push the needle straight down completely into the skin Fig. 7. 24. Keep the needle all the way in the skin while slowly pushing the syringe plunger down until the barrel is empty Fig. 8. Release the pinched skin and pull the needle straight out. Dispose of the needle and syringe in the sharps container without recapping or removing the needle.

After the injection

25. Do not rub the injection area. If bleeding occurs, apply a clean, dry cotton swab over the area, and press gently for 1 to 2 minutes, or until bleeding stops Fig. 9. Then apply an adhesive plaster. 26. Safely dispose of needles and syringe in the sharps container or as directed by your healthcare provider or pharmacist Fig. 10. Never reuse syringes or needles. 27. Properly dispose of vials containing remaining water and Ilaris solution if any as directed by your healthcare provider or pharmacist. Any unused product or waste material should be disposed of in accordance with local requirements. Keep the sharps container out of reach of children. Dispose of it as directed by your healthcare provider or pharmacist.