Anakinra for Tocilizumab-Refractory Febrile Infection-Related Epilepsy Syndrome (FIRES) with Normal IL-1β Levels: A Case Report - Report - MDSpire

Anakinra for Tocilizumab-Refractory Febrile Infection-Related Epilepsy Syndrome (FIRES) with Normal IL-1β Levels: A Case Report

  • By

  • Zhang, Lijun

  • Li, Deyuan

  • Wang, Jianjun

  • Wang, Xiao

  • Zhao, Wanling

  • Lu, Guoyan

  • June 1, 2026

  • 0 min

Share

Clinical Report: Anakinra in Febrile Infection-Related Epilepsy Syndrome

Overview

This case report highlights the successful use of anakinra in a pediatric patient with febrile infection-related epilepsy syndrome (FIRES) who was resistant to tocilizumab. Despite normal IL-1β levels, anakinra administration led to rapid seizure control and improved clinical outcomes.

Background

Febrile infection-related epilepsy syndrome (FIRES) is a severe condition characterized by super-refractory status epilepticus following a febrile illness, with limited treatment options available. Neuroinflammation, particularly involving cytokines like IL-1β and IL-6, plays a critical role in the pathophysiology of FIRES. The sequential use of IL-6 receptor blockade followed by IL-1 receptor blockade is not well-documented, especially in cases with normal IL-1β levels.

Data Highlights

No numerical data available in the article.

Key Findings

  • Anakinra was effective in controlling seizures in a child with FIRES resistant to tocilizumab.
  • The patient had normal IL-1β levels, challenging the assumption that elevated IL-1β is necessary for anakinra efficacy.
  • Initial treatment with tocilizumab showed temporary improvement, but seizures relapsed, necessitating a switch to anakinra.
  • The patient achieved seizure control within five days of starting anakinra and was discharged with mild cognitive dysfunction.
  • At one-month follow-up, the patient experienced rare brief seizures and was able to attend school.

Clinical Implications

Discuss potential risks or considerations for clinicians when using anakinra in patients with normal IL-1β levels.

Conclusion

This case underscores the importance of considering anakinra for refractory FIRES, highlighting its potential effectiveness regardless of IL-1β biomarker status.

Related Resources & Content

  1. Frontiers in Immunology, 2026 -- Case Report: Tocilizumab in pediatric febrile infection-related epilepsy syndrome
  2. Frontiers in Pediatrics, 2026 -- Respiratory Effects of Off label Anakinra in Critically Ill Neonates: A 15-Patient Observational Cohort Study
  3. Clinical Rheumatology, 2008 -- Efficacy and Safety of Anakinra for Treating Polyarticular Juvenile Rheumatoid Arthritis: Findings from a Randomized Multicenter Trial
  4. International NORSE Consensus Recommendations Published, NORSE INSTITUTE -- 2022
  5. Observational Study of Tocilizumab in Children With Febrile Infection‐Related Epilepsy Syndrome - He - 2025 - Annals of Clinical and Translational Neurology
  6. Clinical Rheumatology — Investigation of Antibody Formation Against Anakinra in Patients with Severe CAPS: A Sub-analysis from a Long-term Safety and Efficacy Study
  7. International NORSE Consensus Recommendations Published — NORSE INSTITUTE
  8. Observational Study of Tocilizumab in Children With Febrile Infection‐Related Epilepsy Syndrome - He - 2025 - Annals of Clinical and Translational Neurology - Wiley Online Library
  9. Functional deficiency in endogenous interleukin‐1 receptor antagonist in patients with febrile infection‐related epilepsy syndrome - PMC

Original Source(s)

Related Content