Multilayer concept of autoimmune mechanisms and manifestations in inborn errors of immunity: Relevance for precision therapy

Autoimmunity in inborn errors of immunity (IEI) has a multifactorial pathogenesis and develops upon a genetic predisposition along side gene regulation, environmental modifiers, and infectious triggers. Primarily based on incremental information availability as a consequence of up-front software of omics-technologies, a extra granular and dynamic view of mechanisms and manifestations is warranted. Right here we current a complete novel idea of autoimmunity in IEI that considers a number of layers of interdependent parts and connects 101 causative genes or deletions based on the standard of the allelic variants with 47 molecular pathways and 22 immune effector mechanisms.

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