Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020 Nov 26;8(22):5821-5830.
doi: 10.12998/wjcc.v8.i22.5821.

Usefulness of ultrasonography to assess the response to steroidal therapy for the rare case of type 2b immunoglobulin G4-related sclerosing cholangitis without pancreatitis: A case report

Yuto Tanaka  1 , Kenya Kamimura  2 , Ryota Nakamura  1 , Marina Ohkoshi-Yamada  1 , Yohei Koseki  1 , Takeshi Mizusawa  1 , Satoshi Ikarashi  1 , Kazunao Hayashi  1 , Hiroki Sato  1 , Akira Sakamaki  1 , Junji Yokoyama  1 , Shuji Terai  1
Affiliations
Case Reports

Usefulness of ultrasonography to assess the response to steroidal therapy for the rare case of type 2b immunoglobulin G4-related sclerosing cholangitis without pancreatitis: A case report

Yuto Tanaka et al. World J Clin Cases. .

Abstract

Background: A type 2b immunoglobulin G4 (IgG4)-related sclerosing cholangitis (SC) without autoimmune pancreatitis is a rare condition with IgG4-SC. While the variety of the imaging modalities have tested its usefulness in diagnosing the IgG4-SC, however, the usage of ultrasonography for the assessment of the response to steroidal therapy on the changes of bile duct wall thickness have not been reported in the condition. Therefore, the information of our recent case and reported cases have been summarized.

Case summary: We report the case of an 82-year-old Japanese man diagnosed with isolated IgG4-related SC based on the increase of serum IgG4, narrowing of the bile duct, its wall thickness, no complication of autoimmune pancreatitis, and IgG4 positive inflammatory cell infiltration to the wall with the fibrotic changes. The cholangiogram revealed type 2b according to the classification. Corticosteroid treatment showed a favorable effect, with the smooth decrease in serum IgG4 and the improvement of the bile duct wall thickness.

Conclusion: As isolated type 2b, IgG4-SC is rare, the images, histological findings, and clinical course of our case will be helpful for physicians to diagnose and treat the new cases appropriately.

Keywords: Autoimmune pancreatitis; Case report; Corticosteroid; Imaging; Immunoglobulin G4-related sclerosing cholangitis; Type 2b; Ultrasonography.

PubMed Disclaimer

Conflict of interest statement

Conflict-of-interest statement: The authors declare that they have no current financial arrangement or affiliation with any organization that may have a direct influence on their work.

Figures

Figure 1
Figure 1
Contrast-enhanced computed tomography showed stricture and mild dilatation of intrahepatic bile ducts and its wall thickening with an enhance effect (A, orange arrows). No significant swelling of the pancreas and the dilatation of main pancreatic duct were observed (B, orange arrow); C: Abdominal ultrasonography and D: Endoscopic ultrasonography showed thickening of the bile duct wall (C and D, orange arrows) and stenosis of the bile duct (C and D, orange arrowheads) in the lower bile duct. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography revealed stenosis of the lower bile duct (E and F, blue arrowheads) and intrahepatic bile ducts (E and F, blue arrows).
Figure 2
Figure 2
Histopathological findings. A tissue sample was collected from the stenotic lower bile duct and stained with hematoxylin and eosin staining (A), IgG (B), IgG4 (C). Marked infiltration of the inflammatory cells (A-C, orange arrowheads) and storiform fibrosis (A-C, orange arrows) were observed. An increase in the number of IgG- (B) and IgG4-positive cells (C) was noted. Liver tissue showed infiltration of inflammatory cells (D: hematoxylin-eosin staining; E: IgG; F: IgG4, orange arrowheads) partly positive for IgG (E) and IgG4 (F). The scale bars represent 100 µm and 50 µm in the insets.
Figure 3
Figure 3
Clinical course. The orange two-direction arrows indicate the wall thickness determined by abdominal ultrasonography. Orange arrowheads indicate the bile duct. The blue arrowhead indicates the endoscopic nasobiliary drainage tube. BD: Bile duct; PSL: Prednisolone; ENBD: Endoscopic nasobiliary drainage; IgG4: Immunoglobulin G4; ALT: Alanine aminotransferase; ALP: Alkaline phosphatase.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Nakazawa T, Naitoh I, Hayashi K, Okumura F, Miyabe K, Yoshida M, Yamashita H, Ohara H, Joh T. Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol. 2012;47:79–87. - PubMed
    1. Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015;385:1460–1471. - PubMed
    1. Inoue D, Yoshida K, Yoneda N, Ozaki K, Matsubara T, Nagai K, Okumura K, Toshima F, Toyama J, Minami T, Matsui O, Gabata T, Zen Y. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore) 2015;94:e680. - PMC - PubMed
    1. Tanaka A, Tazuma S, Okazaki K, Nakazawa T, Inui K, Chiba T, Takikawa H. Clinical Features, Response to Treatment, and Outcomes of IgG4-Related Sclerosing Cholangitis. Clin Gastroenterol Hepatol 2017; 15: 920-926. :e3. - PubMed
    1. Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, Topazian MD, Clain JE, Pearson RK, Petersen BT, Vege SS, Lindor K, Farnell MB. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–715. - PubMed

Publication types