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

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Clinical Scorecard: Anakinra Administration in a Case of Febrile Infection-Related Epilepsy Syndrome (FIRES) Resistant to Tocilizumab with Normal IL-1β Levels

At a Glance

CategoryDetail
ConditionFebrile Infection-Related Epilepsy Syndrome (FIRES)
Key MechanismsNeuroinflammation involving IL-1β and IL-6
Target PopulationChildren with super-refractory status epilepticus following febrile infections
Care SettingPediatric critical care

Key Highlights

  • FIRES is a severe epileptic encephalopathy with limited treatment options.
  • The patient initially improved with tocilizumab but experienced seizure relapse.
  • Anakinra led to rapid seizure control within five days.
  • The patient was discharged with mild cognitive dysfunction.
  • Anakinra may be considered as a second-line option in selected patients.

Guideline-Based Recommendations

Diagnosis

  • Diagnosis of FIRES based on clinical presentation and history of febrile infection.

Management

  • Initial treatment with tocilizumab followed by anakinra in refractory cases.

Monitoring & Follow-up

  • Monitor seizure activity and cognitive function post-treatment.

Risks

  • Potential for cognitive dysfunction and super-refractory status epilepticus.

Patient & Prescribing Data

A 6-year-old boy with super-refractory status epilepticus.

Anakinra was effective despite normal IL-1β levels.

Clinical Best Practices

  • Consider anakinra for patients with FIRES unresponsive to tocilizumab.
  • Evaluate cytokine levels to guide treatment decisions.

Related Resources & Content

    Original Source(s)

    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

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