Abstract
IgG4‐related sclerosing cholangitis (IgG4‐SC) is the biliary manifestation of IgG4‐related disease, systemic fibroinflammatory condition that is characterized by lesions with classical histopathological findings in involved organs. We presented a case of isolated IgG4‐SC with the normal serum IgG4 which was hard to differentiate with cholangiocarcinoma.
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