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Treatment response to Janus kinase inhibitor in a child affected by <scp>Aicardi‐Goutières</scp> syndrome

Jessica Galli, Marco Cattalini, Erika Loi, Rosalba Monica Ferraro, Silvia Giliani, Simona Orcesi, Lorenzo Pinelli, Raffaele Badolato, Elisa Fazzi

2023Clinical Case Reports10 citationsDOIOpen Access PDF

Abstract

Key Clinical Message Baricitinib, a Janus kinase inhibitor (JAK‐inhibitor), seems to contribute to an improvement of a child affected by Aicardi‐Goutières syndrome (AGS), reducing the interferon score and determining a recovery of cognitive, communicative, and relational dysfunctions, while the gross motor deficit persisted. Abstract We report the treatment response to baricitinib, a JAK‐inhibitor, in a 4‐year‐old girl affected by Aicardi‐Goutières syndrome (AGS2, RNASEH2B mutation). Using quantitative measures, we detected a significant amelioration characterized by a complete recovery of cognitive, communicative, and relational skills after 8 and 16 months from the beginning of therapy.

Topics & Concepts

MedicineJanus kinase 3Janus kinaseCognitionPediatricsInternal medicineImmunologyReceptorPsychiatryImmune systemAntigen-presenting cellT cellinterferon and immune responsesInflammasome and immune disordersAutoimmune and Inflammatory Disorders Research
Treatment response to Janus kinase inhibitor in a child affected by <scp>Aicardi‐Goutières</scp> syndrome | Litcius