Among patients with IgA nephropathy (IgAN), kidney failure develops in ≥30% of cases within 20 to 30 years, despite optimized standard care. A critical step in the pathogenesis of IgAN is the production of galactose-deficient IgA1(Gd-IgA1), which triggers autoantibody release. Historically, interventions for IgAN have focused on supportive treatments, such as renin–angiotensin system inhibitors. With an improved understanding of disease pathogenesis, treatments can be categorized according to the ‘four-hit hypothesis,’ targeting different stages of the process. Therapeutic strategies are now divided into non-immunologic (e.g., ACEi/ARBs, Sodium-glucose cotransporter-2 (SGLT2) inhibitors, sparsentan) and immunologic therapies(e.g., corticosteroids, Nefecon, APRIL/BAFF inhibition, complement blockade).Immunologic therapy in IgAN has traditionally been limited to glucocorticoids, which are associated with significant adverse effects. In December 2023, an oral, delayed-release formulation of budesonide (Nefecon) became the first drug to receive full approval from the U.S. Food and Drug Administration (FDA) for the treatment of IgAN. Sparsentan is a dual endothelin and angiotensin II receptor antagonist that has been shown to reduce proteinuria and slow kidney function decline in adults with IgAN who are at risk of disease progression. Atrasentan, a selective endothelin type A receptor antagonist currently being evaluated in a phase 3 clinical trial, has also demonstrated a significant reduction in proteinuria at 36 weeks. This review summarizes key studies on the treatment of IgA nephropathy (IgAN),

which is now entering a new therapeutic era.