- 期刊
- OpenAccess
Unmasking IgG4-related autoimmune pancreatitis from pancreatic cancer: A lesson learned
摘要
Introduction: Autoimmune pancreatitis (AIP) is difficult to distinguish from pancreatic cancer. An AIP has a similar clinical presentation and is one component of a systemic disease, Immunoglobulin G4 (IgG4) related sclerosing disease. IgG4 related sclerosing disease is characterized by extensive IgG4 positive plasma cells and T-lymphocytes that may involve the pancreas and other organs. Case Report: A 77-year-old male presented with nausea, vomiting, decreased appetite, and 39 lbs weight loss over three months. An endoscopic ultrasound showed one 3.3x2.7 cm mass on the tail of the pancreas extending towards the splenic hilum with no lymph node involvement. A fine needle aspiration was indeterminate. The patient underwent distal pancreatectomy and splenectomy for presumed pancreatic cancer, however, the pathology report was negative for malignancy. Instead, the microscopic examination revealed dense lymphoplasmacytic infiltrate staining positive for IgG4, onion-skin pattern fibrosis, and lobular atrophy in the medium sized pancreatic duct that was consistent with AIP. Conclusion: IgG4 related sclerosing disease is a systemic disorder that may involve multiple organs. The clinical manifestations are similar to that of pancreatic malignancy. For this reason, diagnostic evaluation to differentiate the two disease entities is crucial, as treatments differ significantly. Treatment for AIP consists of early initiation with glucocorticoid therapy. However, poor response to treatment usually indicates advanced fibrotic changes or an alternative diagnosis. Failure to differentiate autoimmune pancreatitis from pancreatic cancer can lead to unnecessary surgery of the pancreas along with complications associated with surgery.