Clinical and Image Characteristics of IgG4-Related Sclerosing Cholecystitis

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.

IgG4-related Sclerosing Disease of the Gallbladder: Incidental Malignancy Mimicker Associated With Cholelithiasis?

“…By imaging, gallbladder carcinomas present as deep hypoechoic areas, an appearance that overlaps with that of IgG4-RD. 24 There is no specific imaging study established to make a definitive diagnosis of IgG4-related cholecystitis; it has been reported as a useful tool for the presence of a layered pattern and the identification of Rokitansky-Aschoff sinuses in the gallbladder to differentiate this entity with gallbladder carcinoma. 25 In previously reported cases, the gallbladder mucosa usually appeared grossly atrophic, with marked wall thickening, a smooth inner surface, and severe adhesions surrounding the specimen, accompanied-when present-by a well-defined, solitary mass ranging from 2 to 6 cm in dimension.…”

“…However, the thickened wall is not a specific finding, as this finding is also present in adenomyomatosis, chronic cholecystitis caused by cholelithiasis, XGC, and carcinoma. By imaging, gallbladder carcinomas present as deep hypoechoic areas, an appearance that overlaps with that of IgG4-RD 24 . There is no specific imaging study established to make a definitive diagnosis of IgG4-related cholecystitis; it has been reported as a useful tool for the presence of a layered pattern and the identification of Rokitansky-Aschoff sinuses in the gallbladder to differentiate this entity with gallbladder carcinoma 25 .…”

New Developments in the Ultrasonography Diagnosis of Gallbladder Diseases

“…IgG4-CC is an emergent organ manifestation of IgG4-related disease, and only a few cases are reported in the literature [89].…”

“…At US examination, the main finding of IgG4-CC is the thickening of the gallbladder wall, which can be diffuse or localized [25]. Recently, a further classification of IgG4-CC wall thickening has been proposed in Japan due to the wider diffusion of IgG4-related diseases [89].…”

“…Sometimes, it can be challenging to differentiate IgG4-CC from GBC [89,90]. For this reason, the important role of CEUS in IgG4-CC is to exclude malignancy (see the specific section) [89].…”