Article
Tocilizumab reduces the unmanageable inflammatory reaction of a patient with Aicardi-Goutières syndrome type 7 during treatment with ruxolitinib.
Pediatric rheumatology online journal - 12 Oct 2023
Wang Wei, Wang Wei, Peng Siming, Gao Sihao, Quan Meiying, Gou Lijuan, Wang Changyan, Sun Zhixing, Li Zhuo, Lian Dongmei, Song Hongmei
Abstract excerpt
BACKGROUND: Aicardi-Goutières syndrome (AGS) is a rare hereditary early-onset encephalopathy characterized by upregulation of the type I interferon pathway, poorly responsive to conventional immunosuppression. CASE PRESENTATION: We describe a 7-year-old Chinese boy who developed symptoms at the age of 6 months. He presented with a chilblain-like rash, leukopenia, neutropenia, elevated liver enzymesgrowth...
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