A 64-year-old man was referred to our hospital after an endoscopic retrograde biliary drainage (ERBD) tube was inserted for obstructive jaundice due to hilar bile duct stenosis. Although initial examinations suggested hilar cholangiocarcinoma with elevated tumor marker (CEA, CA19-9), the bile duct stenosis relieved during precise examination. Together with mild elevation of serum IgG4, IgG4-related sclerosing cholangitis was highly suspected. The follow-up ERCP one month later, however, showed re-stenosis of hilar bile duct and FDG-PET/CT demonstrated moderate accumulations at the stenotic site, gallbladder wall and the lymph node in the hepatoduodenal ligament. We therefore decided to perform the operation: the cut end of the cystic duct and dissected lymph nodes in the hepatoduodenal ligament showed cancer cell negative, and the cholecystectomy alone was performed. The gallbladder had characteristic histological features of hyalinizing cholecystitis (HC) and IgG4-related disease. Finally, we diagnosed IgG4-related sclerosing cholangitis associated with HC. To the best of our knowledge, this is the first case which can be referred to an association between IgG4-related sclerosing cholangitis and HC.
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