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Aicardi-Goutières syndrome (AGS) is a rare early-onset auto-inflammatory disease characterized by basal ganglia calcification, chronic cerebrospinal fluid (CSF) lymphocytosis, and elevated type I interferon (IFN) levels in the CSF (1, 2). Typical clinical manifestations include developmental delay, intellectual impairment, chilblain, panniculitis, glaucoma, and autoimmunity overlapping with systemic lupus erythematosus (SLE) This article is protected by copyright. All rights reserved.

Efficacy of Baricitinib in the Treatment of Chilblains Associated With Aicardi‐Goutières Syndrome, a Type I Interferonopathy