Infection and autoinflammation in inborn errors of immunity: brothers in arms

•Various components of immune signaling pathways can regulate inflammatory responses and host defense.•Autoinflammation and increased susceptibility to infection present together in many inborn errors of immunity.•In-depth studies of inborn errors of immunity are likely to reveal novel therapeutic avenues. The binary view of inborn errors of immunity classified as either autoinflammatory conditions or primary immunodeficiency in the strict sense, that is, increased susceptibility to infection is challenged by the description of recent inborn errors of immunity (IEI) triggers leading to activation and disruption of cell death pathways, play a major part in the pathophysiology of infection and autoinflammation. In addition, molecules with a double role in the extracellular versus intracellular milieu add to the complexity. In all, in-depth study of human inborn errors of immunity will continue to instruct us on fundamental immunology and lead to novel therapeutic targets and approaches that can be used in other monogenic and polygenic/complex immune disorders.