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

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

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