Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis

Autor: Hiroshi Nishida, Jiro Fujimoto, Etsuro Hatano, Hayato Miyamoto, Takahiro Ueki, Seikan Hai, Kenjiro Iida, Kazuhiro Suzumura, Keiji Nakasho, Tatsuya Andoh, Masaharu Tada, Hideaki Sueoka, Kentaro Nonaka
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Zdroj: Case Reports in Gastroenterology
Case Reports in Gastroenterology, Vol 12, Iss 2, Pp 425-431 (2018)
ISSN: 1662-0631
Popis: A 75-year-old male was admitted to our hospital because of bile duct stenosis. He had no medical history of autoimmune disease. The level of tumor markers, serum IgG, and IgG4 were within normal ranges. Computed tomography showed perihilar and distal bile duct stenosis and wall thickening without swelling or abnormal enhancement of the pancreas. Endoscopic retrograde cholangiopancreatography showed perihilar and distal bile duct stenosis. A biopsy and cytology from the distal bile duct stenosis suggested adenocarcinoma, and cytology from the perihilar bile duct also suggested adenocarcinoma. A preoperative diagnosis of perihilar and distal bile duct cancer was made, and the patient underwent left hepatectomy and pancreaticoduodenectomy. Resected specimens showed wall thickening in the perihilar and distal bile duct; however, tumors were unclear. A histopathological examination revealed lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis in the perihilar and distal bile ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the perihilar and distal bile ducts. Lymphoplasmacytic infiltration, inflammatory change, storiform fibrosis, and obliterative phlebitis were shown in the pancreas. A final diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC) with autoimmune pancreatitis was made. We herein report a case in which a preoperative diagnosis of IgG4-SC was difficult due to normal serum IgG4 levels and no obvious pancreatic lesion.
Databáze: OpenAIRE