Freeman et al. provide practical advice on prevention, diagnosis, and management of infections in inborn errors of immunity as well as management of common complications such as bronchiectasis.

Martins-Ferreira and Ballestar review how ICF syndrome serves as a paradigm of epigenetic immunodeficiency. They show how alterations in DNA methylation and chromatin remodeling disrupt genome stability and B cell maturation, highlighting broader principles linking epigenetic regulation to immunity and informing other primary immunodeficiencies.

This review synthesizes human inborn errors of the alternative NF-κB pathway-affecting core components, upstream ligands/receptors, or regulators, or regulatory elements-highlighting their shared and distinct effects on lymphoid development, immune dysregulation, and mTEC-mediated tolerance, which can lead to impaired B and T cell function, the development of neutralizing anti-type I IFN autoantibodies, susceptibility to severe viral diseases, and syndromic features.