Abstract

Inflammatory involvement of the bile duct and pancreas can present in different ways depending on the etiology and place of involvement. These are rare diseases; the most common is autoimmune pancreatitis, which has two subtypes: type 1, which is considered the pancreatic manifestation of systemic IgG4-related disease (IgG4-RD) and type 2, which is considered a primarily pancreatic disease. The most common clinical presentation is in the form of jaundice and pancreatic masses with or without abdominal pain, which necessitates a differential diagnosis with pancreatic adenocarcinoma. Treatment is based on corticosteroids, with good response rates, but there is a non-negligible risk of recurrence which sometimes requires treatment with anti-CD20 (rituximab) or immunosuppressants. IgG4-RD can also affect the bile duct, in which case it is called IgG4-related sclerosing cholangitis, and treatment is similarly based on corticosteroids. Lastly, recurrent pyogenic cholangitis is a rare type of inflammatory disease of the bile duct characterized by the formation of recurrent intrahepatic and extrahepatic bile duct stones and recurrent episodes of cholangitis that often require endoscopic, radiological, or surgical treatment in addition to medical treatment.

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