For NOMID | About KINERET® (anakinra)
She’s one of a kind. So is her treatment.
KINERET is the first and only approved treatment for neonatal-onset multisystem inflammatory disease (NOMID).1Prescribe KINERET
NOMID is a rare autoinflammatory disease.
Belonging to a group of disorders known as cryopyrin-associated periodic syndromes (CAPS), NOMID is the most severe. Early diagnosis and treatment is critical to preventing the long-term, irreversible damage and disability that can occur.2-4
NOMID can be challenging to diagnose.
- A diagnosis is derived from inflammatory markers, clinical symptoms, severity, and age of onset2,3
- Symptoms of NOMID include aseptic meningitis, urticaria-like rash, fever, vomiting, joint pain, headache, and conjunctivitis1,3
- Over time, chronic inflammation can lead to developmental delay, sensorineural hearing loss, physical disability, and intellectual disability2,3
- Although NOMID is often associated with mutations in the CIAS1/NLRP3 gene, approximately 40% of NOMID patients test negative using conventional genetic analyses5
- 2016 clinical diagnostic criteria for CAPS do not “mandate evidence of a disease-causing NLRP3 mutation”3
NOMID is driven by interleukin-1.
- Interluekin-1 (IL-1α and IL-1β) is a prototypic proinflammatory cytokine
- Secretion of IL-1β has an important role in systemic inflammation and in the signs and symptoms of NOMID1,6,7
- NOMID is often associated with mutations in the CIAS1/NLRP3 gene, which encodes the protein cryopyrin (NLRP3), an important component of the NLRP3 inflammasome*8,9
- Although approximately 40% of patients test negative for CIAS1/NLRP3 using conventional genetic analyses, advanced testing techniques have identified somatic NLRP3 mosaicism in ~70% of these patients9
- A mutation in cryopyrin leads to an increased rate of inflammasome assembly without the necessary inflammatory stimuli2,9
- Increased inflammasome activity results in excessive production of the pro-inflammatory cytokine IL-1β2,8,10
* A multiprotein complex that activates inflammatory responses
KINERET blocks both IL-1α and IL-1β.
- KINERET, a recombinant IL-1 receptor antagonist (IL-1Ra), blocks the biologic activity of both IL-1α and IL-1β by inhibiting IL-1 binding to the IL-1 type 1 receptor (IL-1R1), which is expressed in a wide variety of tissues and organs1
- KINERET supplements naturally occurring endogenous IL-1Ra, working in a similar way to block the IL-1 receptor without removing endogenous IL-1 from the circulation6