Immunoglobulin G4 (IgG4)-related disease is a systemic fibro-inflammatory condition characterized by tumefactive lesions, dense lymphoplasmacytic infiltration, and storiform fibrosis. Among its various presentations, IgG4-related digestive system disorders (IgG4-DSD) are the most prevalent, frequently mimicking malignancies and posing significant diagnostic challenges. This review synthesizes advances over the past five years in the clinical spectrum, pathogenesis, and biomarker landscape of IgG

