Immunoglobulin G4 ‐related gastrointestinal disease associated with type 1 autoimmune pancreatitis: A case report

Author:

Sato Shingo1ORCID,Kasai Toyotaka1,Eto Hiroyuki1,Okamoto Shikiko12,Nagashima Ariki1,Ushiyama Rui1,Shimazaki Reiri3,Nitta Hiroshi3,Arai Motonori4,Ito Hiroshi1

Affiliation:

1. Department of Gastroenterology Fukaya Red Cross Hospital Saitama Japan

2. Department of Gastroenterology Kumagaya General Hospital Saitama Japan

3. Department of Surgery Fukaya Red Cross Hospital Saitama Japan

4. Department of Diagnostic Pathology Fukaya Red Cross Hospital Saitama Japan

Abstract

AbstractImmunoglobulin G4 (IgG4)‐related diseaseis a systemic inflammatory condition of unknown etiology characterized by increases in serum IgG4 and in the number of IgG4‐positive cells in affected tissues. One of the commonly involved locations is the pancreas; this condition is known as type 1 autoimmune pancreatitis (AIP). Type 1 AIP, which shows a biliary stricture in the intrapancreatic bile duct, can be misdiagnosed as a malignancy due to similar cholangiography findings and clinical presentation. In rare cases complicated by post‐bulbar duodenal ulcers, differentiating between type 1 AIP and malignancies is even more difficult. An 81‐year‐old male was referred to our hospital for the treatment of a pancreatic head mass and obstructive jaundice. Serological and radiological findings were consistent with both type 1 AIP and a malignancy. Gastroduodenoscopy revealed a post‐bulbar duodenal ulcer with endoscopic features that evoked malignant duodenal invasion. Although biopsies were negative for malignant cells, subsequent bleeding from the lesion suggested the progression of malignancy, which led to surgical resection. Pancreatoduodenectomy and pathological examination indicated that type 1 AIP was present. Simultaneously, the involvement of IgG4‐related disease in the ulcerative lesion was suggested. To our knowledge, this is the first reported case of type 1 AIP complicated by post‐bulbar duodenal ulcers, which was misdiagnosed as malignancy and considered an IgG4‐related gastrointestinal disease associated with type 1 AIP.

Publisher

Wiley

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