Hilar cholangiocarcinoma associated with immunoglobulin G4‐positive plasma cells and elevated serum immunoglobulin G4 levels
Nabeel Azeem, Veeral Ajmera, Bilal Hameed, Neil Mehta – 28 February 2018 – Immunoglobulin G4 (IgG4)‐related disease is a fibroinflammatory systemic disorder with multiorgan involvement. Proximal bile duct involvement results in IgG4‐related sclerosing cholangitis, which is characterized by a lymphoplasmacytic infiltrate with abundant IgG4‐positive plasma cells and fibrosis. Differentiating between cholangiocarcinoma and IgG4‐sclerosing cholangitis can present a diagnostic dilemma.