[Autoimmune pancreatitis and IgG4-related disease].

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated systemic disease that causes organ-specific inflammation and fibrosis patterns and also frequently has tumor-like effects. In the pancreaticobiliary setting type 1 autoimmune pancreatitis (AIP1) and IgG4-related cholangitis (IAC) are the most important manifestations. Structured diagnostics are decisive, with prioritized exclusion of malignancies and infections followed by a syndrome-based classification (serology, imaging, histology and organ pattern). Glucocorticoids are highly effective for induction of remission; however, recurrences are frequent and require a risk-adapted prevention of recurrence and structured monitoring of the course.