فهرست نشریات علمی دسترسی آزاد ایران

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition that can affect multiple organs, most notably the pancreas, where it manifests as autoimmune pancreatitis (AIP). Necrotizing pancreatitis is an extremely rare presentation of IgG4-RD. We report the case of a 73-year-old man with type 2 diabetes mellitus and cardiovascular comorbidities who presented with persistent abdominal pain, weight loss, and laboratory findings of leukocytosis, anemia, and elevated inflammatory markers. Imaging studies revealed pancreatic necrosis, vascular thrombosis, and peripancreatic inflammation. Hematologic evaluation excluded multiple myeloma despite abnormal free light chain levels, supporting immune dysregulation secondary to IgG4-RD. Elevated serum IgG4 (165.2 mg/dL) along with marked clinical improvement after corticosteroid pulse therapy established the diagnosis of IgG4-related autoimmune pancreatitis. This case highlights the diagnostic challenge of differentiating AIP from malignancy and plasma cell dyscrasias, the importance of considering IgG4-RD in necrotizing pancreatitis, and the pivotal role of steroid responsiveness in confirming the diagnosis. To our knowledge, this is the first reported case of IgG4-related necrotizing pancreatitis associated with vascular complications and hematologic abnormalities.