Abstract
IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.
Highlights
Immunoglobulin G4-related sclerosing cholangitis (IgG4SC) is a relatively uncommon but increasingly recognized entity
AST, ALT, and bilirubin levels were within normal limits, and Alkaline phosphatase (ALP) was elevated at 127 IU/L. e computed tomography (CT) scan of abdomen revealed intrahepatic biliary ductal dilatation and gall bladder wall thickening with pericholecystic inflammatory stranding with normal pancreas (Figure 1)
E differentiation of IgG4-related sclerosing cholangitis (IgG4-Sclerosing cholangitis (SC)) from primary sclerosing cholangitis (PSC) and cholangiocarcinoma is clinically very important as IgG4-SC usually has a good response to steroid therapy [10]
Summary
Immunoglobulin G4-related sclerosing cholangitis (IgG4SC) is a relatively uncommon but increasingly recognized entity It was first described in 2001 by Hamano et al [1] with a landmark study demonstrating elevated levels of serum IgG4 in patients with sclerosing cholangitis [1,2,3,4]. It is characterized by systemic inflammatory and sclerosing lesions with massive infiltration of IgG4-positive lymphocytes involving multiple organ systems, such as the eyes, salivary glands, lacrimal glands, lungs, pancreas, kidneys, retroperitoneum, and vascular system [2, 5]. Isolated IgG4-SC can occur rarely and pose a diagnostic challenge [5, 6]
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