Abstract
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by the infiltration of IgG4-positive plasma cells in affected tissues, leading to fibrosis and progressive organ dysfunction. This review explores the epidemiology, pathogenesis, and organ manifestations of IgG4-RD, with a focus on autoimmune pancreatitis and sclerosing cholangitis as the main clinical presentations. It may cause exocrine and endocrine pancreatic insufficiency and chronic hepatobiliary failure. Main diagnostic challenges include differentiation from malignancies and other inflammatory conditions. Diagnosis of IgG4-RD involves combination of clinical symptoms, typical imaging findings, elevated serum IgG4 levels, and histopathological evidence of IgG4-positive plasma cell infiltration. Advances in clinical understanding of the disease, histopathological and serological markers, imaging techniques, have enhanced early detection. Current treatment strategies prioritize steroids therapy for induction of remission, while steroid-sparing agents, including disease-modifying antirheumatic drugs and rituximab play the pivotal roles in managing its relapses or steroid-resistant disease. Biologic therapies are also promising therapeutic avenues. In addition, multidisciplinary approach optimizes the diagnosis, treatment, and long-term outcomes in this complex disease.