For DIRA | About Kineret® (anakinra)

DIRA is ultra-rare. With Kineret, his hope doesn't have to be.

Kineret has been demonstrated to be effective for the treatment of deficiency of interleukin-1 receptor antagonist (DIRA).1

Baby looking at camera, being held by mother

DIRA is an ultra-rare pediatric autoinflammatory disease.2

DIRA is an autosomal recessive disorder caused by mutations in the IL1RN gene, leading to loss of production and function of the interleukin-1 receptor antagonist (IL-1Ra).2

  • 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 antibiotics2
  • DIRA has a 25% mortality rate, and there are currently no known patients who, without treatment, have reached adulthood2,4
Baby looking at camera

Learn more about Kineret

Kineret is 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 interleukin-1.

  • Interleukin-1 (IL-1α and IL-1β) is a prototypic proinflammatory cytokine4
    • Secretion of IL-1 has an important role in systemic inflammation and in the signs and symptoms of DIRA1,4
       
  • DIRA is an autosomal recessive monogenic autoinflammatory disease caused by mutations in the IL1RN gene, leading to loss of function and secretion of interleukin-1 receptor antagonist (IL-1Ra)1
     
  • The deficiency of IL-1Ra results in unopposed IL-1α and IL-1β proinflammatory signaling, causing severe systemic inflammation with skin and bone involvement1
Kineret blocks the signal to stop inflammation

Kineret blocks unopposed IL-1 proinflammatory signaling to relieve DIRA symptoms.

  • Kineret is the recombinant form of IL-1Ra that DIRA patients lack. It supplements naturally occurring endogenous IL-1Ra and works in a similar way1,4
  • Kineret blocks the biologic activity of IL-1α and IL-1β by competitively inhibiting IL-1 binding to the interleukin 1 type I receptor (IL-1RI), which is expressed in a wide variety of tissues and organs1
See the Data