ANSWERS TO YOUR
QUESTIONS
ABOUT
CAPS AND ILARIS

What are CAPS?

Cryopyrin-Associated Periodic Syndromes (CAPS) are a group of rare genetic diseases that includes Familial Cold Autoinflammatory Syndrome (FCAS) and Muckle-Wells Syndrome (MWS). CAPS are rare and chronic diseases that are part of a larger group of diseases known as Periodic Fever Syndromes.

What causes CAPS?

CAPS are inherited conditions that are generally passed on from other family members. CAPS are caused by an inherited mutated gene, called the NLRP3 gene (or the CIAS1 gene). In some cases, however, the genetic mutation responsible for CAPS may occur spontaneously at birth.

How long do CAPS last?

There is no cure for CAPS, but with continuous treatment, patients with CAPS may experience symptom improvement.

What are Periodic Fever Syndromes?

Periodic Fever Syndromes are a group of rare, autoinflammatory diseases that are characterized by symptoms including recurrent fevers, rash, pain, and joint inflammation. Periodic Fevers are typically inherited from family members, but can occur on their own. Both children and adults can be affected by Periodic Fevers, though symptoms usually begin during childhood.

Are CAPS contagious?

CAPS are not contagious. They're a group of inherited diseases that can be passed down from family members, though they can also occur on their own. Certain factors are thought to trigger CAPS flares, including exposure to cold, drops in temperature, stress, and exercise.

What is ILARIS?

ILARIS^® is a prescription medication that is FDA approved to treat CAPS, including FCAS and MWS, in patients 4 years and older. ILARIS works by attaching to and blocking interleukin-1 beta (IL-1β), a type of cytokine responsible for inflammation. For CAPS, ILARIS is given every 8 weeks as a subcutaneous injection right below the skin.

How can ILARIS help with CAPS?

ILARIS specifically addresses inflammation caused by IL-1β that can lead to CAPS symptoms such as rash, joint and/or muscle pain, and eye irritation. ILARIS can also decrease the risk of flares, or the worsening of CAPS symptoms. ILARIS is given by a doctor or nurse as an injection right below the skin. Talk to a doctor to find out if ILARIS may be a treatment option for you or your child.

How fast does ILARIS work?

Seven days after just 1 dose of ILARIS, the majority of patients (71%) experienced a complete response* to treatment. 97 percent of patients experienced improvement in their CAPS symptoms within the first treatment period (8 weeks).

*In a clinical study of 35 patients, 97% had a complete response within the first treatment period (8 weeks). The majority of patients (71%) experienced a complete response to treatment in 7 days.

Complete response was measured by laboratory tests and a doctor's evaluation of the patients' skin disease and symptoms being minimal or better, including symptoms such as: rash, fatigue, muscle pain, headache or migraine, sore or red eyes, and joint pain.

Is ILARIS given intravenously?

ILARIS is not given as an intravenous infusion (infused inside a vein over a period of time). Instead, it is given as a subcutaneous (right under the skin) injection, or shot, by a doctor or nurse. A home health nurse can be sent to your home to administer ILARIS. If you are eligible, this service and other ILARIS support options may be available for you or your child.

How often is ILARIS given?

For CAPS, ILARIS is given as a subcutaneous injection (right under the skin) by a doctor or nurse once every 8 weeks—just 6 or 7 treatments in a year.

Is support available to help us pay for ILARIS?

We're committed to helping you get access to ILARIS. Our ILARIS Companion representatives are here to help by working with your doctor or your child's doctor, and:

Contacting your insurance company
Verifying insurance benefits and investigation of coverage
Addressing coverage issues
Navigating prior authorization and appeals, if needed
Providing co-pay* assistance for eligible patients with commercial insurance
Providing information about alternative assistance options for uninsured patients

*Limitations apply. Please contact ILARIS Companion at 866-972-8315 for more information.

Whom should I speak to if I have more questions about ILARIS?

Talk to a rheumatologist or other healthcare professional. An active CAPS treatment team can consist of many important members. If you or your child has CAPS, make sure to find a doctor that works for you.

Next: CAPS Glossary

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IMPORTANT SAFETY INFORMATION

ILARIS can cause serious side effects, including increased risk of serious infections. ILARIS can lower the ability of your immune system to fight infections. Your healthcare provider should:

test you for tuberculosis (TB) before you receive ILARIS
monitor you closely for symptoms of TB during treatment with ILARIS
check you for symptoms of any type of infection before, during, and after treatment with ILARIS

Tell your healthcare provider right away if you have any symptoms of an infection such as fever, sweats or chills, cough, flu-like symptoms, weight loss, shortness of breath, blood in your phlegm, sores on your body, warm or painful areas on your body, diarrhea or stomach pain, or feeling very tired.

Do not receive ILARIS if you are allergic to canakinumab or any of the ingredients in ILARIS.

Before receiving ILARIS, tell your healthcare provider about all your medical conditions, including if you:

think you have or are being treated for an active infection
have symptoms of infection
have a history of infections that keep coming back
have a history of low white blood cells
have or have had HIV, Hepatitis B, or Hepatitis C
have recently received or are scheduled to receive any vaccinations
- You should be brought up to date with all age required vaccines before starting treatment with ILARIS
- You should not receive "live" vaccines while you are being treated with ILARIS and until your healthcare provider tells you that your immune system is no longer weakened

APPROVED USE

ILARIS^® (canakinumab) is a prescription medicine injected by your healthcare provider just below the skin (subcutaneous) used to treat:

The following autoinflammatory Periodic Fever Syndromes:
- Cryopyrin-Associated Periodic Syndromes (CAPS) in patients 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) also known as Mevalonate Kinase Deficiency (MKD) in adult and pediatric patients
- Familial Mediterranean Fever (FMF) in adult and pediatric patients
Still's disease including Adult-Onset Still's Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA) in children 2 years of age and older
Gout flares in adults who:
- are not able to receive or tolerate treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) and colchicine
- have not responded to treatment with NSAIDs and colchicine
- are not able to receive repeated treatment with steroids

It is not known if ILARIS is safe and effective when used to treat:

SJIA in children under 2 years of age
CAPS in children under 4 years of age
gout flares in children

are pregnant or planning to become pregnant. It is not known if ILARIS will harm your unborn baby. Tell your healthcare provider right away if you become pregnant while receiving ILARIS
received ILARIS while you were pregnant. It is important that you tell your baby's healthcare provider before any vaccinations are given to your baby within 4 to 12 months after you received your last dose of ILARIS before giving birth
are breastfeeding or planning to breastfeed. It is not known if ILARIS passes into your breast milk. Talk to your healthcare provider about the best way to feed your baby if you receive ILARIS

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements. Especially tell your healthcare provider if you take:

medicines that affect your immune system
medicines called interleukin-1 (IL-1) blocking agents such as Kineret^® (anakinra) or Arcalyst^® (rilonacept)
medicines called tumor necrosis factor (TNF) inhibitors such as Enbrel^® (etanercept), Humira^® (adalimumab), Remicade^® (infliximab), Simponi^® (golimumab), or Cimzia^® (certolizumab pegol)
medicines that affect enzyme metabolism

Ask your healthcare provider for a list of these medicines if you are not sure.

ILARIS can cause serious side effects including:

serious infections
decreased ability of the body to fight infections (immunosuppression). For people treated with medicines that cause immunosuppression like ILARIS, the chances of getting cancer may increase
allergic reactions. Allergic reactions can happen while receiving ILARIS. Call your healthcare provider right away if you have any of these symptoms of an allergic reaction: difficulty breathing or swallowing, nausea, dizziness or feeling faint, rash, itching or hives, palpitations (feels like your heart is racing), or low blood pressure
risk of infection with live vaccines. You should not get live vaccines if you are receiving ILARIS. Tell your healthcare provider if you are scheduled to receive any vaccines

The most common side effects of ILARIS when used for the treatment of CAPS include: cold symptoms, diarrhea, flu (influenza), runny nose, headache, cough, body aches, nausea, vomiting, and diarrhea (gastroenteritis), feeling like you are spinning (vertigo), weight gain, injection-site reactions (such as redness, swelling, warmth, or itching), and nausea.

The most common side effects of ILARIS when used for the treatment of TRAPS, HIDS/MKD, and FMF include: cold symptoms, upper respiratory tract infection, runny nose, sore throat, nausea, vomiting, and diarrhea (gastroenteritis), and injection-site reactions (such as redness, swelling, warmth, or itching).

The most common side effects of ILARIS when used for the treatment of Still's disease (AOSD and SJIA) include: cold symptoms, upper respiratory tract infection, pneumonia, runny nose, sore throat, urinary tract infection, nausea, vomiting, and diarrhea (gastroenteritis), stomach pain, and injection-site reactions (such as redness, swelling, warmth, or itching).

The most common side effects of ILARIS when used for the treatment of gout flares include: cold symptoms, upper respiratory tract infection, urinary tract infection, increased levels of triglycerides in blood, and back pain.

Tell your healthcare provider about any side effect that bothers you or does not go away.

What is Macrophage Activation Syndrome (MAS)?

MAS is a syndrome associated with Still's disease and some other autoinflammatory diseases like HIDS/MKD that can lead to death. Tell your healthcare provider right away if your AOSD or SJIA symptoms get worse or if you have any of these symptoms of an infection:

a fever lasting longer than 3 days
a cough that does not go away
redness in one part of your body
warm feeling or swelling of your skin

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.1-800-FDA-1088.

Please see full Prescribing Information, including Medication Guide.

All trademarks and registered trademarks are the property of their respective owners.

APPROVED USE

ILARIS^® (canakinumab) is a prescription medicine injected by your healthcare provider just below the skin (subcutaneous) used to treat:

The following auto-inflammatory Periodic Fever Syndromes
- Cryopyrin-Associated Periodic Syndromes (CAPS) in patients 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) also known as Mevalonate Kinase Deficiency (MKD) in adult and pediatric patients
- Familial Mediterranean Fever (FMF) in adult and pediatric patients
Still's disease including Adult-Onset Still's Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (SJIA) in children 2 years of age and older.
Gout flares in adults who:
- are not able to receive or tolerate treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) and colchicine
- have not responded to treatment with NSAIDs and colchicine
- are not able to receive repeated treatment with steroids

It is not known if ILARIS is safe and effective when used to treat SJIA in children under 2 years of age or when used to treat CAPS in children under 4 years of age or when used to treat gout flares in children.

ANSWERS TO YOUR QUESTIONS ABOUT CAPS AND ILARIS

ANSWERS TO YOUR
QUESTIONS
ABOUT
CAPS AND ILARIS