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Refractory cerebral autoinflammatory-autoimmune diseases are often associated with dysregulated innate immunity and are targeted by anakinra, an interleukin-1 receptor antagonist. We analyzed the therapeutic effect of anakinra in refractory cerebral autoinflammatory response (CAIR) at a single institution from January 2017 to May 2021. In total, 12 patients with various etiologies were sympathetically treated with anakinra (100 mg/day subcutaneously). Four patients showed good responses, and among these patients, three patients had pathologically demonstrated CAIR. The other eight patients were nonresponsive. No patient had a serious adverse effect. Thus, anakinra may be a therapeutic option for refractory cerebral autoinflammatory diseases. © 2022 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.

Citation

Yoonhyuk Jang, Woo-Jin Lee, Han Sang Lee, Kon Chu, Sang Kun Lee, Soon-Tae Lee. Anakinra treatment for refractory cerebral autoinflammatory responses. Annals of clinical and translational neurology. 2022 Jan;9(1):91-97

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PMID: 35040583

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