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Multiple intrahepatic artery aneurysms during the treatment for IgG4-related sclerosing cholangitis: A case report.

World J Hepatol

December 2024

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki 889-1692, Japan.

Article Synopsis
  • The case report discusses a 64-year-old woman with IgG4-related sclerosing cholangitis who developed multiple intrahepatic artery aneurysms while on treatment.
  • Emergency procedures successfully treated the aneurysms, resolving her symptoms and stabilizing her condition.
  • The report highlights that hepatic artery aneurysms can be serious complications in patients with IgG4-SC, indicating a need for vigilant monitoring.
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A 60-year-old male was admitted for obstructive jaundice secondary to a 2 cm retroperitoneal lesion. Ultrasound endoscopy (UES) with fine needle biopsy (FNB) was performed, as well as endoscopic retrograde cholangiopancreatography (ERCP) with placement of a metal stent for bile duct drainage. Initially IgG4-related disease was suspected from FNB.

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Since the concept of IgG4-related disease (IgG4-RD) was proposed, that diagnosis has been considered in idiopathic fibroinflammatory diseases in various organs, particularly in cases with multi-organ involvement. We have recently encountered three cases of fibrosing disease of uncertain etiology with shared microscopic appearances. Case 1 (56-year-old man) had an irregular mass at the base of mesentery.

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Objectives: The objective of this study was to examine the imaging features of hepatic inflammatory pseudotumors (IPTs) associated with IgG4-related and IgG4-unrelated conditions and to enhance the approach toward distinguishing between these two types of IPTs.

Methods: A retrospective study was conducted, involving 20 patients diagnosed with hepatic IPTs. Imaging procedures were conducted within a timeframe of 4 weeks prior to hepatectomy or biopsy.

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A 60-year-old female with a BRCA2 mutation and a history of breast cancer presented with diffuse abdominal pain and elevated liver enzymes. Imaging revealed a porta-hepatis mass, prompting consideration of hilar cholangiocarcinoma or breast cancer metastasis. Further investigation including biopsy and F-fluorodeoxyglucose positron emission tomography/computed tomography findings were inconsistent with malignancy, leading to investigation of non-neoplastic causes.

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