Abstract:The IgG4-related disease is an uncommon immune-mediated condition affecting multiple organ systems concomitantly; however, it is very rare for a patient to have isolated IgG4 autoimmune cholangitis or IgG4related disease confined to the hepatobiliary system. The majority of cases are associated with pancreatitis and are incidentally discovered in the workup of acute or chronic pancreatitis. When it affects the hepatobiliary system, it develops as inflammatory fibrosclerosing cholangitis, which can mimic other … Show more
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