title: Autoimmune pancreatitis type-1 associated with intraduct papillary mucinous neoplasm: report of two cases.
abstract: Chronic pancreatitis lesions usually embrace both intraduct papillary mucinous neoplasm (IPMN) and pancreatic ductal adenocarcinoma (PDAC). Patients at genetically-determined high risk of PDAC often harbor IPMN and/or chronic pancreatitis, suggesting IPMN, chronic pancreatitis and PDAC may share pathogenetic mechanisms. Chronic autoimmune pancreatitis (AIP) may also herald PDAC. Concurrent IPMN and AIP have been reported in few patients. Here we describe two patients with IPMN who developed type-1 AIP fulfilling the Honolulu and Boston diagnostic criteria. AIP diffusively affected the whole pancreas, as well as peripancreatic lymph nodes and the gallbladder. Previous pancreatic resection of focal IPMN did not show features of AIP. One of the patients carried a CFTR class-I mutation. Of notice, serum IgG4 levels gradually decreased to normal values after IPMN excision. Common risk factors to IPMN and AIP may facilitate its coincidental generation.
authors: Department of Gastroenterology, Institut de Malalties Digestives i Metabòliques, Hospital Clínic, CIBEREHD, IDIBAPS, Barcelona, Spain.
authors: Eva C
authors: EC
authors: Vaquero
authors: Department of Pathology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors: Maria T
authors: MT
authors: Salcedo
authors: Department of Pathology, Centre de Diagnòstic Biomèdic (CDB), Hospital Clínic, University of Barcelona and Banc de Tumors-Biobanc Clinic-IDIBAPS-XBTC, Barcelona, Spain.
authors: Míriam
authors: M
authors: Cuatrecasas
authors: Exocrine Pancreatic Diseases Research Group, Hospital Universitari Vall d'Hebron, Institut de Recerca (VHIR), Universitat Autònoma de Barcelona, CIBEREHD, Barcelona, Spain.
authors: Hannah
authors: H
authors: De León
authors: Department of Radiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors: Xavier
authors: X
authors: Merino
authors: Department of Gastroenterology, Institut de Malalties Digestives i Metabòliques, Hospital Clínic, CIBEREHD, IDIBAPS, Barcelona, Spain.
authors: Salvador
authors: S
authors: Navarro
authors: Department of Gastroenterology, Institut de Malalties Digestives i Metabòliques, Hospital Clínic, CIBEREHD, IDIBAPS, Barcelona, Spain.
authors: Angels
authors: A
authors: Ginès
authors: Department of Endoscopy, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors: Monder
authors: M
authors: Abu-Suboh
authors: Department of Surgery, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors: Joaquim
authors: J
authors: Balsells
authors: Department of Surgery, Institut de Malalties Digestives i Metabòliques, Hospital Clínic, CIBEREHD, IDIBAPS, Barcelona, Spain.
authors: Laureano
authors: L
authors: Fernández-Cruz
authors: Exocrine Pancreatic Diseases Research Group, Hospital Universitari Vall d'Hebron, Institut de Recerca (VHIR), Universitat Autònoma de Barcelona, CIBEREHD, Barcelona, Spain. Electronic address: xavier.molero@vhir.org.
authors: Xavier
authors: X
authors: Molero
mesh_terms: D000231:Adenocarcinoma, Papillary / Q000150:complications*; D000368:Aged; D001327:Autoimmune Diseases / Q000209:etiology*; D018282:Cystadenocarcinoma, Mucinous / Q000209:etiology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D010190:Pancreatic Neoplasms / Q000150:complications*; D050500:Pancreatitis, Chronic / Q000209:etiology*; D012307:Risk Factors
chemical_list: D007074:Immunoglobulin G; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Autoimmune pancreatitis; CFTR; Chronic pancreatitis; Diagnostic criteria; IPMN; IgG4