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What is DIRA?
Dosing and Administration
Step By Step Administration
Treatment Resources
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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.1,2 Patients with DIRA have life-threatening systemic inflammation with skin and bone involvement.1 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.2,3 

DIRA is a very rare disease with fewer than 50 patients worldwide.4 Cases have been identified in families originating in Puerto Rico, Newfoundland (Canada), The Netherlands, Palestine/Lebanon, Germany, Turkey, India, and Brazil.2,5,6 In Puerto Rico, the incidence of DIRA in some regions may be as high as 1 in 6300 births.1 Although difficult to estimate, mortality is approximately 30% in early infancy.4 

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.1,2,5 Generalized skin pustulosis, joint swelling, and painful osteolytic lesions are some of the more distinctive clinical features of the disease.2 

Children with DIRA experience intense chronic pain.7 This pain may lead to feeding challenges and subsequent growth delays.7 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.4,5

ARCALYST is indicated for the maintenance of remission of DIRA in adults and pediatric patients weighing at least 10 kg.8

DOSING AND ADMINISTRATION 

ARCALYST is a patient-administered once-weekly subcutaneous therapy.8

The once-weekly maintenance dose can be self-administered by the patient.


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 maximum at 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 3 mL

STEP BY STEP ADMINISTRATION

ARCALYST reconstitution and injection follow a step-by-step process.8

ARCALYST is supplied in sterile, single-use, 20-mL glass vials.8

  • Each vial contains 220 mg rilonacept, a sterile, white to off-white, preservative-free, lyophilized powder
  • Reconstitution with 2.3 mL of Sterile Water for Injection is required prior to subcutaneous administration of the drug
  • The reconstituted ARCALYST is a viscous, clear, colorless to pale yellow, free from particulates, 80-mg/mL solution

 

Step 1
PRE-ENROLLMENT

  • Ensure your patient’s vaccination history is up to date, including their pneumonia and flu vaccines.

Step 2
ENROLLMENT FORM COMPLETION

  • The Enrollment Form will be provided by your Kiniksa Sales Specialist or is available for download below. 
  • Fax completed Enrollment Form to Kiniksa OneConnect at (781) 609-7826.

Step 3
FULFILLMENT

  • Your patient will be contacted by Kiniksa OneConnect to help arrange delivery from select specialty pharmacies. 
  • Kiniksa OneConnect will work with your patients to set up one-on-one injection training sessions with either virtual support from an ARCALYST Clinical Educator or a combination of virtual/in-person training support that includes virtual support from an ARCALYST Clinical Educator and in-person support from a healthcare professional.
Enrollment Form (English)

Kiniksa OneConnect can help your patients with their treatment needs.

TREATMENT RESOURCES 

There may be several steps to obtaining approval for your patient’s treatment with ARCALYST.  The downloadable resources below were developed to help simplify this process by providing information about gaining access to ARCALYST, understanding reimbursement claims, and working with a specialty pharmacy.

Access 
The following downloadable resources can help guide you through the steps to product access.

Financial Assistance  
Resources for information about access to ARCALYST and navigating the reimbursement process.

KINIKSA ONECONNECT™

Kiniksa OneConnect™ is a support program made up of a team of experienced individuals, known as Patient Access Leads, with knowledge of insurance plans and healthcare networks.

Patient Access Leads

Once enrolled in Kiniksa OneConnect™, your patient will be paired with a dedicated Patient Access Lead who will work to provide personalized one-on-one support throughout your patient's entire treatment experience.

ARCALYST Clinical Educators

ARCALYST Clinical Educators can conduct sessions to help provide your patients with training on the injection process.

Beginning treatment

By helping to navigate your patients through their insurance coverage and partnering with your practice, we make getting your patient on treatment a seamless experience in a variety of ways:

  • We coordinate, verify, and explain the benefits verification process
  • We assist with the prior authorization and appeals processes if required
  • When it's time for your patients to begin treatment, we help coordinate delivery of your patient’s therapy

Financial assistance

Our programs help identify possible sources of financial support for eligible patients.

  • The Kiniksa Copay Assistance Program can offer copays as low as $0 per month*
  • The Quick Start Program supports qualified patients with delay in coverage for treatment initiation while awaiting prior authorization†
  • The Patient Assistance Program supports qualified patients with no coverage for treatment‡
  • To be eligible for the Kiniksa Copay Assistance Program, your patients must have commercial insurance, must not have Medicare, Medicaid, or other government insurance, and must meet other eligibility criteria. Your patient also must agree to the rules set forth in the terms and conditions for the program. Please visit kiniksapolicies.com/copay to review additional eligibility criteria.
  • Program offered for up to 60 days. To be eligible for the Kiniksa Quick Start Program, your patient must meet certain financial eligibility requirements. Please visit kiniksapolicies.com/qstart to review additional eligibility criteria. 
  • Program offered for up to 12 months. To be eligible for the Kiniksa Patient Assistance Program, your patient must meet certain financial eligibility requirements. Please visit kiniksapolicies.com/pap to review additional eligibility criteria.

    • Treatment support

    We provide access to Registered Nurses and Licensed Practical Nurses who are trained in both disease state and ARCALYST administration to maximize the patient experience. There are multiple injection training options including:

    • In-office training by you or your office staff
    • One-on-one injection training sessions with an ARCALYST Clinical Educator

    Help your patients learn more about our services.

    Download

    Call 1-833-KINIKSA (1-833-546-4572), Option 1 
    Monday-Friday (8 AM – 8 PM ET)

     References: 1. Aksentijevich I, Masters SL, Ferguson PJ, et al. An autoinflammatory disease with deficiency of the interleukin-1 receptor antagonist. N Engl J Med. 2009;360(23):2426-2437. 2. Lachmann HJ. Periodic fever syndromes. Best Pract Res Clin Rheumatol. 2017;31(4):596-609. 3. Autosomal dominant inheritance. National Cancer Institute Dictionary of Genetics Terms. Accessed February 23, 2021. https://www.cancer.gov/publications/dictionaries/genetics-dictionary/def/autosomal-dominant-inheritanc 4. Garg M, de Jesus AA, Chapelle D, et al. Rilonacept maintains long-term inflammatory remission in patients with deficiency of the IL-1 receptor antagonist. JCI Insight. 2017;2(16):e94838. 5. Dyall-Smith D. Deficiency of the interleukin-1 receptor antagonist. DermNet NZ. https://dermnetnz.org/topics/deficiency-of-the-interleukin-1-receptor-antagonist-dira/ 6. Gómez-García F, Sanz-Cabanillas JL, Viguera-Guerra I, Isla-Tejera B, Vélez-García Nieto AV, Ruano J. Scoping review on use of drugs targeting interleukin 1 pathway in DIRA and DITRA. Dermatol Ther (Heidelb). 2018;8(4):539-556. 7. Deficiency of IL-1 receptor antagonist (IL-1RA) known as DIRA. Autoinflammatory Alliance. Accessed February 23, 2021. http://autoinflammatory.org/dira.php 8. ARCALYST. Package insert. Kiniksa Pharmaceuticals. 

    Request to speak to a Clinical Sales Specialist

    Indication

    ARCALYST® (rilonacept) is an interleukin-1 blocker indicated for:

    • Treatment of recurrent pericarditis (RP) and reduction in risk of recurrence in adults and pediatric patients 12 years and older.
    • Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS), including Familial Cold Auto-inflammatory Syndrome (FCAS), and Muckle-Wells Syndrome (MWS) in adults and children 12 years and older. 
    • Maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in adults and pediatric patients weighing 10 kg or more.

    Important Safety Information

    Warnings and Precautions

    • Interleukin-1 (IL-1) blockade may interfere with the immune response to infections. Treatment with another medication that works through inhibition of IL-1 or inhibition of tumor necrosis factor (TNF) is not recommended as this may increase the risk of serious infection. Serious, life-threatening infections have been reported in patients taking ARCALYST. Do not initiate treatment with ARCALYST in patients with an active or chronic infection.
    • Discontinue ARCALYST if a patient develops a serious infection.
    • It is possible that taking drugs such as ARCALYST that block IL-1 may increase the risk of tuberculosis (TB) or other atypical or opportunistic infections.
    • Although the impact of ARCALYST on infections and the development of malignancies is not known, treatment with immunosuppressants, including ARCALYST, may result in an increase in the risk of malignancies.
    • Hypersensitivity reactions associated with ARCALYST occurred in clinical trials. Discontinue ARCALYST and initiate appropriate therapy if a hypersensitivity reaction occurs. 
    • Increases in non-fasting lipid profile parameters occurred in patients treated with ARCALYST in clinical trials. Patients should be monitored for changes in their lipid profiles.
    • Since no data are available, avoid administration of live vaccines while patients are receiving ARCALYST. ARCALYST may interfere with the normal immune response to new antigens, so vaccines may not be effective in patients receiving ARCALYST. It is recommended that, prior to initiation of therapy with ARCALYST, patients receive all recommended vaccinations, as appropriate.

    Adverse Reactions

    • In patients with CAPS or RP, the most common adverse reactions (≥10%) include injection-site reactions and upper respiratory tract infections.
    • In patients with DIRA, the most common adverse reactions (>10%) include upper respiratory tract infections, rash, otitis media, pharyngitis, and rhinorrhea.

    Drug Interactions

    • In patients being treated with CYP450 substrates with narrow therapeutic indices, therapeutic monitoring of the effect or drug concentration should /pi.pdfbe performed, and the individual dose of the medicinal product may need to be adjusted.

    For more information about ARCALYST, see full Prescribing Information.