Background. Autoimmune pancreatitis (AIP) often mimics pancreatic cancer. The diagnosis of both conditions is difficult preoperatively let alone when they coexist. Several reports have been published describing pancreatic cancer in the setting of AIP. Case Report. The case of a 53-year-old man who presented with abdominal pain, jaundice, and radiological features of autoimmune pancreatitis, with a "sausage-shaped" pancreas and bulky pancreatic head with portal vein impingement, is presented. He had a normal serum IgG4 and only mildly elevated Ca-19.9. Initial endoscopic ultrasound-(EUS-) guided fine-needle aspiration (FNA) of the pancreas revealed an inflammatory sclerosing process only. A repeat EUS guided biopsy following biliary decompression demonstrated both malignancy and features of autoimmune pancreatitis. At laparotomy, a uniformly hard, bulky pancreas was found with no sonographically definable mass. A total pancreatectomy with portal vein resection and reconstruction was performed. Histology revealed adenosquamous carcinoma of the pancreatic head and autoimmune pancreatitis and squamous metaplasia in the remaining pancreas. Conclusion. This case highlights the diagnostic and management difficulties in a patient with pancreatic cancer in the setting of serum IgG4-negative, Type 2 AIP.
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http://dx.doi.org/10.1155/2013/809023 | DOI Listing |
Gastroenterol Clin North Am
March 2025
Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA. Electronic address:
Autoimmune pancreatitis (AIP) is a steroid-responsive fibroinflammatory disorder with 2 clinically distinct subtypes known as type 1 autoimmune and type 2 autoimmune pancreatitis. Type 1 AIP is considered the pancreatic manifestation of immunoglobulin G4-related disease, a systemic disease often presenting with other organ involvement. Advances in understanding the unique clinical presentation, imaging findings, histopathology, and clinical course of this relatively uncommon disease have led to international consensus regarding diagnosis and treatment.
View Article and Find Full Text PDFEndosc Ultrasound
December 2024
Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China.
Background And Objectives: EUS is recommended for guiding pancreatic tissue acquisition in suspected autoimmune pancreatitis (AIP) cases. However, there is a lack of comparative research on the effectiveness between EUS-guided fine-needle aspiration (EUS-FNA) and EUS-guided fine-needle biopsy (EUS-FNB) for diagnosing AIP in China. This study aimed to evaluate the diagnostic accuracy of EUS-guided tissue acquisition (EUS-TA) specifically for type 1 AIP.
View Article and Find Full Text PDFInt J Surg Pathol
January 2025
Department of Laboratory Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
IgG4-related disease (IgG4-RD) is an autoimmune disease of unknown cause. is a transcription factor involved in immune responses, and its dysfunction leads to uncontrolled immune responses. We performed, to our knowledge, the first methylation analysis in type 1 autoimmune pancreatitis (denoted simply as AIP), a representative IgG4-RD.
View Article and Find Full Text PDFCureus
December 2024
Nephrology, Unidade Local de Saúde de São José, Lisbon, PRT.
Immunoglobulin G4-related disease (IgG4-RD) and systemic lupus erythematosus (SLE) are multisystemic autoimmune disorders that can present with renal manifestations. Overlapping cases of these diseases are extremely rare and present both diagnostic and therapeutic challenges. We report the case of a 70-year-old male with a history of autoimmune pancreatitis, who was admitted with fatigue, weight loss, and worsening kidney function.
View Article and Find Full Text PDFTherap Adv Gastroenterol
January 2025
Department of Gastroenterology, Peking University First Hospital, No. 8 Xishiku Street, Xicheng District, Beijing 100034, China.
Background: Inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn's disease (CD), can affect the hepatobiliary system and pancreas, substantially impacting the life quality of patients.
Objectives: To evaluate the quality of evidence and comprehensively assess the validity of associations of IBD with hepatobiliary and pancreatic diseases.
Design: We performed an umbrella review of existing meta-analyses in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) recommendations.
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