title: A Patient with Autoimmune Pancreatitis Type 1 with Previously Known Lymphadenopathy, Both in the Context of IgG4-related Disease.
abstract: 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.
  
  
  METHODS
  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.
authors.affiliation: Department of Internal Medicine, Erasmus Medical Centre, Rotterdam, Netherlands.
authors.forename: Fazil M
authors.initials: FM
authors.lastname: Alidjan
authors.affiliation: Department of Internal Medicine, Erasmus Medical Centre, Rottterdam, Netherlands.
authors.forename: Faiz
authors.initials: F
authors.lastname: Karim
authors.affiliation: Department of Pathology, Erasmus Medical Centre, Rotterdam, Netherlands.
authors.forename: Rob M
authors.initials: RM
authors.lastname: Verdijk
authors.affiliation: Department of Internal Medicine and Gastroenterology, Amphia Hospital, Breda, Netherlands.
authors.forename: Joost W
authors.initials: JW
authors.lastname: van Esser
authors.affiliation: Department of Internal Medicine and Gastroenterology, Amphia Hospital, Breda, Netherlands.
authors.forename: Marianne J
authors.initials: MJ
authors.lastname: van Heerde
mesh_terms: D000888:Antibodies, Anti-Idiotypic / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology*; D001706:Biopsy; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008206:Lymphatic Diseases / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D050500:Pancreatitis, Chronic / Q000175:diagnosis / Q000276:immunology*; D014057:Tomography, X-Ray Computed
chemical_list: D000888:Antibodies, Anti-Idiotypic; D007074:Immunoglobulin G