Objective: Unusual clinical course Background: Autoimmune pancreatitis (AIP) is an important clinical pathologic concept of IgG-4-related disease. AIP is a rare cause of chronic pancreatitis, characterized by a fibroinflammatory process by lymphoplasmacytic infiltrates, storiform fibrosis, obliterative phlebitis, and increased IgG4+ plasma cells, leading to dysfunction of the pancreas. Affected patients with AIP frequently have disease affecting other organs or sites with similar histologic changes, elevated IgG4+ plasma cell infiltrate, and good response to corticosteroid therapy. These diseases often are not limited to the pancreas and the pancreas may not be involved at all. Case Report: We report a 62-year-old man with obstructive jaundice with pre-existent submandibular lymphadenopathy. Diagnosis of AIP was based on diagnostic criteria by the HISORT-criteria in combination with elevated IgG-4 serum levels. CT revealed a focal enlargement of the head of the pancreas, as well as mesenteric peripancreatic and mediastinal lymphadenopathy. He was treated with high-dose steroid in combination with azathioprine and showed good clinical response. Conclusions: We report a case with pre-existent submandibular lymphadenopathy and obstructive jaundice based on AIP type 1, both in the context of IgG4-related disease.

Chronic, Digestive system abnormalities, Immunoglobulin G, Pancreatitis
dx.doi.org/10.12659/AJCR.895059, hdl.handle.net/1765/82270
American Journal of Case Reports
Department of Internal Medicine

Alidjan, F.M, Karim, A.F, Verdijk, R.M, van Esser, J.W.J, & van Heerde, M. (2015). A patient with autoimmune pancreatitis type 1 with previously known lymphadenopathy, both in the context of igG4-related disease. American Journal of Case Reports, 16, 790–793. doi:10.12659/AJCR.895059