Issues

Human immunity is often insufficient against infectious agents and sometimes over-reactive to innocuous components of the environment or the human body. Genetic, immunological, and clinical studies of inborn errors of immunity—where responses are either too weak or too strong—offer unprecedented insights into the physiology and pathology of host defense. This is the focus of the Journal of Human Immunity.

Although the pathogenesis of XLA is well understood, several issues remain open for discussion. We describe several unresolved issues, including non-coding BTK variants, contiguous deletion syndrome, Helicobacter infection, non-infectious neurodegeneration, renal involvement, and malignancies.

Here, we report the 2024 update of the phenotypic classification by the International Union of Immunological Societies (IUIS) expert committee on inborn errors of immunity (IEI). This phenotypic classification is presented in the form of decision trees when possible, with essential clinical or immunological phenotype entries.

Here we provide a comprehensive update to the classification of monogenic immune disorders.

Hajjar et al. identify distinct gut microbiome profiles in CVID patients with noninfectious complications and establish a novel germ-free mouse model using fecal microbiota transplant. This model recapitulates human dysbiosis and provides a platform to explore microbiome-driven immune dysregulation in CVID.