Abstract
Immunoglobulin (Ig) G4 (IgG4)-related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. Although best described in cases of autoimmune pancreatitis, IgG4-related disease has also been implicated in patients with cholangitis and is now commonly referred to as IgG4-related cholangiopathy. It is characterized by elevated serum IgG4 levels, an IgG4-positive plasma cell infiltration with storiform fibrosis/obliterative phlebitis of the bile duct wall, and a response to steroids. It is crucial to differentiate IgG4-related cholangiopathy from its mimickers, such as primary biliary cholangitis, secondary biliary cholangitis, primary sclerosing cholangitis, secondary sclerosing cholangitis, and cholangiocarcinoma, because treatment modalities and outcomes of IgG4-related cholangiopathy differ significantly from these disorders. Here, we present an interesting case of IgG4-related cholangiopathy, discuss clinical and pathological features crucial to its early diagnosis, and compare and contrast this condition with its potentially confounding mimickers.
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