Case report examines symptoms, diagnosis, and treatment options for NEMO-NDAS in children, indicating a need for awareness.
NEMO-deleted exon 5 autoinflammatory syndrome (NEMO-NDAS) is the result of a gain-of-function IKBKG pathogenic variant leading to dysregulated NF-κB signaling and systemic inflammation. We present a case of NEMO-NDAS in a 2-year-old female presenting with recurrent fevers, subcutaneous nodules, lymphadenopathy, and splenomegaly. This article highlights the symptoms that aid in diagnosis and how to differentiate it from other autoinflammatory diseases and reviews therapies for NEMO-NDAS.
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Yang et al. (2026) studied this question.
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