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Medications for Interleukin-1 Receptor Antagonist Deficiency

Other names: Deficiency of Interleukin-1 Receptor Antagonist; DIRA

About Interleukin-1 Receptor Antagonist Deficiency

Deficiency of Interleukin-1 Receptor Antagonist (DIRA) is a very rare, genetic, autoinflammatory disease caused by autosomal recessive mutations in the IL1RN gene.

The IL1RN gene produces the interleukin-1 receptor antagonist (IL-1Ra) protein, which plays a critical role in helping regulate the interleukin-1 (IL-1) signaling pathway. IL-1 is a powerful driver of inflammation and plays an important role in the body’s immune response. IL-1 pro-inflammatory signaling primarily occurs through IL-1α and IL-1β. IL-Ra competes with IL-1α and IL-1β, inhibiting pro-inflammatory signaling. In patients with DIRA, the deficiency of the IL-1Ra leads to unopposed action of IL-1α and IL-1β, resulting in life-threatening systemic inflammation with skin and bone involvement.

DIRA usually presents in newborns at birth or in the first few days of life, and the severe inflammatory reaction resembles an acute severe systemic infection, or infection of the bone. As DIRA can be life-threatening, early diagnosis and treatment is essential to prevent death multi-organ failure and death.

Treatments for DIRA include anakinra, an interleukin-1 receptor antagonist (IL-1Ra), and rilonacept, an interleukin-1 alpha (IL-1α) and interleukin-1 beta (IL-1β) cytokine trap.

 

 

Drugs used to treat Interleukin-1 Receptor Antagonist Deficiency

The following list of medications are in some way related to, or used in the treatment of this condition.

Drug name Rating Reviews Activity ? Rx/OTC Pregnancy CSA Alcohol
anakinra Rate Add review
Rx B N

Generic name: anakinra systemic

Brand name:  Kineret

Drug class: antirheumatics, interleukin inhibitors

For consumers: dosage, interactions, side effects

For professionals: A-Z Drug Facts, AHFS DI Monograph

Kineret Rate Add review
Rx B N

Generic name: anakinra systemic

Drug class: antirheumatics, interleukin inhibitors

For consumers: dosage, interactions, side effects

For professionals: Prescribing Information

Arcalyst Rate Add review
Rx C N

Generic name: rilonacept systemic

Drug class: interleukin inhibitors

For consumers: dosage, interactions, side effects

For professionals: Prescribing Information

rilonacept Rate Add review
Rx C N

Generic name: rilonacept systemic

Brand name:  Arcalyst

Drug class: interleukin inhibitors

For consumers: dosage, interactions, side effects

For professionals: A-Z Drug Facts, AHFS DI Monograph

Legend

Rating For ratings, users were asked how effective they found the medicine while considering positive/adverse effects and ease of use (1 = not effective, 10 = most effective).
Activity Activity is based on recent site visitor activity relative to other medications in the list.
Rx Prescription Only.
OTC Over the Counter.
Rx/OTC Prescription or Over the Counter.
Off-label This medication may not be approved by the FDA for the treatment of this condition.
EUA An Emergency Use Authorization (EUA) allows the FDA to authorize unapproved medical products or unapproved uses of approved medical products to be used in a declared public health emergency when there are no adequate, approved, and available alternatives.
Pregnancy Category
A Adequate and well-controlled studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
B Animal reproduction studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
C Animal reproduction studies have shown an adverse effect on the fetus and there are no adequate and well-controlled studies in humans, but potential benefits may warrant use in pregnant women despite potential risks.
D There is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience or studies in humans, but potential benefits may warrant use in pregnant women despite potential risks.
X Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience, and the risks involved in use in pregnant women clearly outweigh potential benefits.
N FDA has not classified the drug.
Controlled Substances Act (CSA) Schedule
N Is not subject to the Controlled Substances Act.
1 Has a high potential for abuse. Has no currently accepted medical use in treatment in the United States. There is a lack of accepted safety for use under medical supervision.
2 Has a high potential for abuse. Has a currently accepted medical use in treatment in the United States or a currently accepted medical use with severe restrictions. Abuse may lead to severe psychological or physical dependence.
3 Has a potential for abuse less than those in schedules 1 and 2. Has a currently accepted medical use in treatment in the United States. Abuse may lead to moderate or low physical dependence or high psychological dependence.
4 Has a low potential for abuse relative to those in schedule 3. It has a currently accepted medical use in treatment in the United States. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 3.
5 Has a low potential for abuse relative to those in schedule 4. Has a currently accepted medical use in treatment in the United States. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 4.
Alcohol
X Interacts with Alcohol.

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Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.