IgG4-Related Disease Mimicking Cholangiocarcinoma
Journal Title: Clinical Radiology & Imaging Journal - Year 2018, Vol 2, Issue 1
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disease that consists of a collection of disorders that share particular pathologic, serologic, and clinical features [1,2]. These disorders were previously thought to be unrelated [3-5]. The most characteristic features include tumor-like swelling of involved organs, a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells, and a variable degree of fibrosis that has a characteristic “storiform” pattern. In addition, elevated serum concentrations of IgG4 are found in 60 to 70 percent of patients with IgG4-RD. IgG4-related sclerosing cholangitis (IgG4-SC) is a characteristic type of sclerosing cholangitis, with an unknown pathogenic mechanism. Patients with IgG4-SC display increased serum IgG4 levels [6] and dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall [7]. Circular and symmetrical thickening of the bile duct wall is observed in the areas without stenosis that appear to be normal on cholangiography, as well as in the stenotic areas [8]. IgG4-SC has been recently recognized as an IgG4-related disease. IgG4-SC is frequently associated with autoimmune pancreatitis (AIP). IgG4-related dacryoadenitis/sialadenitis and IgG4-related retroperitoneal fibrosis are also occasionally present with IgG4-SC [3,10-11]. However, some IgG4-SC cases do not involve other organs [8]. IgG4-SC is most common in elderly men. Obstructive jaundice is frequently observed in IgG4-SC.
Authors and Affiliations
Hala El-Hadary
Keywords
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- EP ID EP565382
- DOI 10.23880/crij-16000117
- Views 62
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