The Food and Drug Administration (FDA) has approved the supplemental Biologics License Application (sBLA) for Kineret® (anakinra; Sobi) for the treatment of deficiency of interleukin-1 receptor antagonist.

Deficiency of interleukin-1 (IL-1) receptor antagonist (DIRA) is an autosomal recessive monogenic autoinflammatory disease caused by a genetic mutation in the IL1RN gene, which leads to the loss of secretion of the IL-1 receptor antagonist (IL-1Ra). The deficiency of IL-1Ra results in unopposed IL-1α and IL-1β pro-inflammatory signaling that causes systemic inflammation with skin and bone involvement. Kineret blocks the biologic activity of IL-1 alpha and beta by competitively inhibiting IL-1 binding to the IL-1 type I receptor.

The approval was based on data from a long-term natural history study that assessed the efficacy and safety of anakinra in 9 patients (ages 1 month to 9 years at the start of treatment) with genetically confirmed DIRA for up to 10 years. Six patients received a starting dose of 1 to 2mg/kg/day (starting dose was not reported in 3 patients), followed by an individually adjusted dose to reach a stable efficacious dose to control active inflammation. At the last visit of the first treatment period, the dose ranged from 2.2 to 6.1mg/kg/day.

Findings showed that all 9 patients achieved inflammatory remission, defined as achievement of all of the following criteria: C-reactive protein (CRP) less than or equal to 5mg/L, no pustulosis, no inflammatory bone disease, and no concomitant glucocorticosteroid use. The study was conducted by investigators at the National Institutes of Health.

The safety profile of anakinra was consistent with that seen in previous studies for patients with neonatal-onset multisystem inflammatory disease (NOMID). The most common adverse reactions reported were upper respiratory tract infections, rash, pyrexia, influenza-like illness and gastroenteritis. There were no permanent discontinuations due to adverse events.

“DIRA signs and symptoms which can occur in the first weeks of life and may be fatal, require urgent diagnosis and management,” said John Yee, MD, MPH, Chief Medical Officer, Sobi North America. “The approval of Kineret for DIRA will allow Sobi to bring a new treatment option to this patient community.”

Kineret is also indicated for the reduction in signs and symptoms and slowing the progression of structural damage in moderately to severely active rheumatoid arthritis in patients age 18 years and older who have failed 1 or more disease modifying antirheumatic drugs (DMARDs). It is also approved for the treatment of NOMID.

The product is available as 100mg/0.67mL single-use prefilled syringes for subcutaneous injection; the graduated syringe allows for doses between 20mg and 100mg.

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  1. FDA approves Kineret® (anakinra) for the treatment of deficiency of il-1 receptor antagonist (DIRA). [press release]. Waltham, MA: Sobi, Inc.; December 22, 2020. 
  2. Kineret [package insert]. Stockholm, Sweden: Sobi, Inc.; 2020.