[Acute cholecystitis associated with IgG4-related sclerosing cholangitis: a case report]

Area of Science:

• Gastroenterology
• Hepatology
• Immunology

Background:

• Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a fibrotic inflammatory condition that can affect the biliary system.
• Distinguishing IgG4-SC from other causes of biliary strictures, such as cholangiocarcinoma, is clinically important.
• IgG4-SC can present with various biliary manifestations, including common bile duct and cystic duct involvement.

Purpose of the Study:

• To report a case of acute cholecystitis secondary to immunoglobulin G4-related sclerosing cholangitis (IgG4-SC).
• To highlight the diagnostic challenges and clinical course of IgG4-SC affecting the biliary tree.
• To demonstrate the efficacy of steroid therapy in managing IgG4-SC-related biliary complications.

Main Methods:

• A 72-year-old male patient presented with jaundice and hilar bile duct stenosis.
• Diagnostic workup included cholangiography, intraductal ultrasonography, and serum immunoglobulin G4 (IgG4) level measurement.
• Endoscopic ultrasonography and cholangiography were used to evaluate acute cholecystitis and cystic duct stenosis.

Main Results:

• Initial findings suggested cholangiocarcinoma but were later revised to IgG4-SC based on elevated IgG4 levels and other organ involvement.
• The patient developed acute cholecystitis with cystic duct stenosis, likely secondary to IgG4-SC.
• Both IgG4-SC and acute cholecystitis showed significant improvement following steroid treatment.

Conclusions:

• IgG4-SC can lead to biliary obstruction and complications like acute cholecystitis.
• Prompt diagnosis and appropriate management, including steroid therapy, are crucial for favorable outcomes in IgG4-SC.
• This case underscores the importance of considering IgG4-SC in the differential diagnosis of biliary strictures and related complications.