Abstract:Immunoglobulin A nephropathy (IgAN) is a common primary glomerular disease characterized by multifaceted, immune-mediated mechanisms. Additionally, several comorbidities, including inflammatory bowel disease, hepatitis, HIV infection, and Sjögren’s syndrome, are intricately linked to the genesis and progression of IgAN and collectively denoted as secondary IgAN. The multiple-hit hypothesis is currently the widely embraced pathogenesis framework for IgAN, positing mucosal dysbiosis-induced disruption of the barrier function and immune aberrations as pivotal triggers for IgAN pathogenesis. The intestinal mucosa, constituting a pivotal element of the mucosal system, is increasingly acknowledged for its substantive involvement in IgAN development. Perturbations in intestinal microbiota, compromised mucosal barrier integrity, and immune dysregulation are recognized as pivotal players in IgAN pathophysiology. Investigating the therapeutic targets associated with these facets currently represents a focal point in research. We comprehensively review the research advances in the roles of intestinal microecology and mucosal immunity in the pathogenesis of IgAN. This review aims to lay a foundation for exploring the novel therapeutic targets for IgAN from intestinal microbiota and immune functionality.