A novel inborn error of immunity perturbs linear ubiquitin homeostasis and causes pyoderma gangrenosum

This session will highlight exciting new insights on human immunology gleaned from the study of monogenic inborn errors of immunity (IEI). IEIs constitute a large group of diverse disorders resulting from genetic defects that compromise innate and adaptive immunity and immune homeostasis. The discovery and characterization of human IEIs has accelerated rapidly in recent years with broadly accessible next generation sequencing technology, comprising >550 distinct disorders according to the 2024 update from the International Union of Immunological Societies. Indeed, the overall incidence of IEIs may be as high as 1 in 1200 people, which may represent an underestimate with many cases still undiagnosed. Importantly, these “experiments of nature” provide significant and often surprising insights on the human immune system that simply cannot be gleaned from animal models alone.The Clinical Immunology Society is dedicated to promoting excellence in IEI-related research and education, training outstanding clinicians and physician scientists, and improving clinical care and advocacy for IEI patients and their families. As interest in human immunology continues to grow in our field, CIS hopes to partner with AAI in showcasing outstanding basic, clinical and translational research on IEIs. Starting with this guest symposium, we hope to foster increased recognition of IEIs and leverage the incomparable expertise of the AAI community in advancing future IEI research.