For DIRA | About Kineret® (anakinra)
NEW INDICATION
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
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
- 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 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