ARCALYST (rilonacept)
For the maintenance of remission of Deficiency of Interleukin 1 (IL-1)
Receptor Antagonist (DIRA)
What is DIRA?
DIRA is an autoinflammatory disease first reported in 2009 that is characterized by the absence of functional interleukin-1 receptor antagonist (IL-1Ra) protein. Patients with DIRA have life-threatening systemic inflammation with skin and bone involvement. DIRA is a genetic disorder inherited in an autosomal recessive manner, meaning that a mutation in both copies of a gene is needed to cause the disease.
DIRA is a very rare disease with fewer than 50 patients worldwide. Cases have been identified in families originating in Puerto Rico, Newfoundland (Canada), The Netherlands, Palestine/Lebanon, Germany, Turkey, India, and Brazil. In Puerto Rico, the incidence of DIRA in some regions may be as high as 1 in 6300 births. Although difficult to estimate, mortality is approximately 30% in early infancy.
DIRA is characterized by a neonatal onset, with patients presenting at birth or in the first days of life with prominent skin and bone manifestations. Some of the more distinctive clinical features of the disease include:
- Large, fluid-filled, blister-like areas of the skin
- Painful areas of damaged bone
- Joint swelling
Children with DIRA experience intense chronic pain. This pain may lead to feeding challenges and subsequent growth delays. If left untreated, DIRA can escalate to life-threatening inflammation, with the development of systemic inflammatory response syndrome (SIRS), multiorgan failure, and death in early childhood.
Please visit the Autoinflammatory Alliance to learn more about DIRA.
About ARCALYST

ARCALYST is indicated for the maintenance of remission of DIRA in adults and pediatric patients weighing at least 10 kg.
Dosing and administration
ARCALYST is a once-weekly
self-administered subcutaneous injection
Your first injection of ARCALYST will be given with the help of a healthcare
provider in the doctor's office, or with support from an ARCALYST Nurse Educator.



Your first injection of ARCALYST will be given with the help of a healthcare provider in the doctor’s office or with support from an ARCALYST Nurse Educator.
Do not try to give yourself ARCALYST injections until you are sure that you understand how to prepare and inject your dose.
The recommended dose for DIRA is shown below.
It is important to use ARCALYST exactly as prescribed by your healthcare provider.
Adults
(18 years and older)
320 mg
injection given once weekly as 2
subcutaneous injections, administered:
- On the same day
- At 2 different sites
- With a maximum single-injection
volume of 3 mL
Pediatric Patients
(weighing at least 10 kg)
4.4 mg/kg
up to a maximum of 320 mg administered
once-weekly as 1-2 subcutaneous
injections. If the dose is given as 2
injections, it should be administered:
- On the same day
- At 2 different sites
- With a maximum single-injection
volume of 2 mL
After training on ARCALYST, you will self-administer a once-weekly injection.
STEP-BY-STEP ADMINISTRATION
Resources to help guide you
In addition to what you have learned from your healthcare provider
or ARCALYST Nurse Educator, the resources below can help you
better understand how to prepare and inject ARCALYST.
