DIRA

KINERET for DIRA

DIRA is ultra-rare. With KINERET, his hope doesn't have to be1

KINERET has been demonstrated to be effective for the treatment of DIRA.1

DIRA

Baby looking at camera, being held by mother

DIRA is an ultra-rare pediatric autoinflammatory disease1

DIRA is an autosomal recessive disorder caused by mutations in the IL1RN gene, leading to loss of production and function of the IL-1Ra.1

  • DIRA generally presents at or near birth and can escalate to life-threatening inflammation2
  • Symptoms of DIRA include swelling of bone tissue, bone pain and deformity, swelling of the tissues around the bones, and skin rash that can cover most of the body3
  • DIRA is often misdiagnosed as infectious osteomyelitis with pustulosis and systemic inflammation, leading to ineffective treatment with antibiotics4
  • DIRA has a 25% mortality rate, and there are currently no known patients who, without treatment, have reached adulthood2
Two overlapping squares that are transparent

A swift diagnosis and initiation of appropriate treatment are essential to improving a DIRA patient’s prognosis.2

Baby looking at camera
Two overlapping squares that are transparent

Learn more about KINERET

KINERET is FDA approved to treat DIRA and in a long-term study was demonstrated to induce inflammatory remission in patients with this painful, life-threatening disease.1

See the data

DIRA is driven by IL-1

  • IL-1 (IL-1α and IL-1β) is a prototypic proinflammatory cytokine2
    • Secretion of IL-1 has an important role in systemic inflammation and in the signs and symptoms of DIRA1,2
  • DIRA is an autosomal recessive monogenic autoinflammatory disease caused by mutations in the IL1RN gene, leading to loss of function and secretion of IL-1Ra1,2
  • The deficiency of IL-1Ra results in unopposed IL-1α and IL-1β proinflammatory signaling, causing severe systemic inflammation with skin and bone involvement1
Learn how KINERET works
Kineret blocks the signal to stop inflammation
Learn how KINERET works
PREVIOUS:   NOMID Case Study
NEXT:   DIRA Efficacy
Important Safety Information
INDICATIONS

KINERET® (anakinra) is an interleukin-1 receptor antagonist indicated for:

Rheumatoid Arthritis (RA). Reduction in signs and symptoms and slowing the progression of structural damage in moderately to severely active rheumatoid arthritis, in patients 18 years of age or older who have failed 1 or more disease-modifying antirheumatic drugs (DMARDs)

Cryopyrin-Associated Periodic Syndromes (CAPS). Treatment of Neonatal-Onset Multisystem Inflammatory Disease (NOMID)

Deficiency of Interleukin-1 Receptor Antagonist (DIRA). Treatment of Deficiency of Interleukin-1 Receptor Antagonist (DIRA)

CONTRAINDICATION

KINERET is contraindicated in patients with known hypersensitivity to E. coli–derived proteins, KINERET, or to any components of the product

IMPORTANT SAFETY INFORMATION

Serious Infections. In RA, discontinue use if serious infection develops. In KINERET-treated NOMID or DIRA patients, the risk of a disease flare when discontinuing KINERET treatment should be weighed against the potential risk of continued treatment. Do not initiate KINERET in patients with active infections

Use in combination with Tumor Necrosis Factor (TNF)-blocking agents is not recommended

Hypersensitivity reactions, including anaphylactic reactions and angioedema, have been reported. Patients with DIRA may have an increased risk of allergic reactions, particularly in the first several weeks after starting KINERET treatment

Immunosuppression. The impact of treatment with KINERET on active and/or chronic infections and the development of malignancies is not known

Immunizations. Live vaccines should not be given concurrently with KINERET

Neutrophil counts should be assessed prior to initiating KINERET treatment, and while receiving KINERET, monthly for 3 months, and thereafter quarterly for a period up to 1 year

Serious Adverse Reactions

RA: The most serious adverse reactions were: Serious Infections and Neutropenia, particularly when used in combination with TNF blocking agents.

NOMID and DIRA: The most serious adverse events were infections.

Most Common Adverse Reactions

RA: The most common adverse reactions (incidence ≥ 5%) are injection site reaction, worsening of rheumatoid arthritis, upper respiratory tract infection, headache, nausea, diarrhea, sinusitis, arthralgia, flu-like symptoms, and abdominal pain

NOMID: The most common AEs during the first 6 months of treatment (incidence > 10%) are injection site reaction, headache, vomiting, arthralgia, pyrexia, and nasopharyngitis

DIRA: The most common AEs are upper respiratory tract infections, rash, pyrexia, influenza-like illness, and gastroenteritis

Post-marketing Experience

Hepato-biliary disorders (elevations of transaminases; non-infectious hepatitis) and thrombocytopenia, including severe thrombocytopenia have been identified during postapproval use of KINERET. Because these reactions are reported voluntarily from a population of uncertain size, it is not always possible to reliably estimate their frequency or establish a causal relationship to drug exposure.

These are not all the possible risks associated with KINERET. Please see Full Prescribing Information for KINERET at https://www.KineretRxHCP.com/home.php
To report suspected adverse reactions, contact Sobi North America at 1-866-773-5274 or FDA at 1-800-FDA-1088
Click here for full Prescribing Information for KINERET.

References: 1. KINERET [Prescribing Information]. Stockholm, Sweden: Swedish Orphan Biovitrum AB (publ). 2. Diaz A. Deficiency of the interleukin-1 receptor antagonist (DIRA). In: Efthimiou P (ed). Auto-Inflammatory Syndromes. Springer, Cham; 2019:69-83. 3. 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. 4. Data on file. Sobi, Inc.