Abstract
Since autoimmune pancreatitis (AIP) was established as a new disease entity, sclerosing change with abundant immunoglobulin-4 (IgG4)-positive plasma cells, storiform fibrosis, and obliterative phlebitis are main pathological features in IgG4-related diseases. Regarding IgG4-related sclerosing cholecystitis (IgG4-CC), which is occasionally associated with AIP cases and is rarely isolated, there are no diagnostic criteria and insufficient perceptions of the image findings. Although there have been some reports on IgG4-CC, differentiation between IgG4-CC and gallbladder cancer is very difficult in some cases with a localized lesion. In this review, we especially focused on image findings of IgG4-CC and summarized its image features for diagnostic assistance. The ultrasonography and CT findings of IgG4-CC could be classified into diffuse and localized types. Based on these findings, the presence of wall thickening with an intact or smooth mucosal layer, followed by a homogenously thickened outer layer, would be a helpful morphological finding to distinguish IgG4-CC from gallbladder cancer.
Highlights
Since autoimmune pancreatitis (AIP) was established as a new entity of immunerelated diseases, namely, immunoglobulin-4 (IgG4)-related sclerosing pancreatitis in 2002, there have been many reports and knowledge regarding AIP and systemic IgG4-related disease accumulated in Japan and around the world
There have been some atypical cases with IgG4-related diseases that were difficult to differentiate from site-specific malignant diseases such as pancreatic cancer, bile duct cancer, lung cancer, submandibular adenocarcinoma, lymphoma, and gallbladder cancer [8]
In the 47 cases, gallbladder stones/debris were not in the cases with AIP, which could be caused by the disturbance of bile flow by the Gn lesion or the misdiagnosis of the gallbladder wall thickening as the secondary change from stones/debris in some cases with AIP, as extrahepatic bile duct or pancreatic head lesions tend to be accompanied by debris
Summary
Since autoimmune pancreatitis (AIP) was established as a new entity of immunerelated diseases, namely, immunoglobulin-4 (IgG4)-related sclerosing pancreatitis in 2002, there have been many reports and knowledge regarding AIP and systemic IgG4-related disease accumulated in Japan and around the world. The fundamental cause and detailed mechanism of IgG4-related disease, except for some candidates of antigens [1,2] and the involvement of B-cells [3,4], have not been clarified, steroid (glucocorticoid) administration has been a gold standard of the therapies, and sclerosing change with abundant IgG4-positive plasma cells (lymphoplasmacytic cells), storiform fibrosis, and obliterative phlebitis are the main pathological features [5,6]. The diagnostic criteria of IgG4-related sclerosing cholangitis have been published, and have indicated that image features such as the specific biliary stricture patterns (type 1–4) and circular and symmetrical thickening of the bile duct wall with a smooth inner margin are characteristic findings [7]. There have been some atypical cases with IgG4-related diseases that were difficult to differentiate from site-specific malignant diseases such as pancreatic cancer, bile duct cancer, lung cancer, submandibular adenocarcinoma, lymphoma, and gallbladder cancer [8]. Among the pathological features of IgG4-related diseases, dense lymphocyte and plasma cell infiltration, a ratio of IgG4-positive plasma cells/IgG-positive cells > 40% and the number of IgG4-positive plasma cells > 10/high powered field, and storiform fibrosis/obliterative phlebitis are common
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