| ISSN |
2307-8960 (online) |
| Open Access |
This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ |
| Copyright |
© The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved. |
| Article Reprints |
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| Permissions |
For details, please visit: http://www.wjgnet.com/bpg/gerinfo/207
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| Publisher |
Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA |
| Website |
http://www.wjgnet.com |
| Category |
Medicine, Research & Experimental |
| Manuscript Type |
Systematic Reviews |
| Article Title |
Primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome associated with inflammatory bowel disease: A case report and systematic review
|
| Manuscript Source |
Invited Manuscript |
| All Author List |
Vinícius Remus Ballotin, Lucas Goldmann Bigarella, Floriano Riva, Georgia Onzi, Raul Angelo Balbinot, Silvana Sartori Balbinot and Jonathan Soldera |
| ORCID |
|
| Funding Agency and Grant Number |
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| Corresponding Author |
Jonathan Soldera, MD, MSc, Associate Professor, Staff Physician, Clinical Gastroenterology, Universidade de Caxias do Sul, Rua Francisco Getúlio Vargas 1130, Caxias do Sul 95084180, Brazil. jonathansoldera@gmail.com |
| Key Words |
Autoimmune hepatitis; Primary sclerosing cholangitis; Crohn’s disease; Ulcerative colitis; Inflammatory bowel diseases; |
| Core Tip |
We have aimed to report a case of a female patient with autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome associated to ulcerative colitis and to systematically review the available cases of AIH and PSC overlap syndrome. A previously healthy 22-year-old woman presented with abdominal pain and jaundice. She had a reagent antinuclear factor (1:640, with a homogeneous nuclear pattern). Magnetic resonance cholangiography showed a cirrhotic liver with multiple focal areas of strictures of the intrahepatic bile ducts, with associate dilations. Liver biopsy described periportal necroinflammatory activity, plasmocyte infiltration, and advanced fibrosis. Colonoscopy had shown ulcerative pancolitis and mild activity (Mayo score 1), with a spared rectum. Treatment with corticosteroids, azathioprine, ursodeoxycholic acid and mesalamine was begun, with improvement in laboratory tests. Searches for the systematic review were run on seven electronic databases, retrieving 3349 references. In the final analysis, 44 references were included, with a total of 109 cases reported. The most common clinical finding was jaundice and 43.5% of cases were associated with inflammatory bowel disease. Among these, 27.6% were cases of Crohn’s disease, 68% of ulcerative colitis, and 6.4% of indeterminate colitis. Most patients were treated with steroids. All-cause mortality was 3.7%. In conclusion, PSC and AIH overlap syndrome is generally associated with inflammatory bowel disease and is associated with low mortality and good response to treatment. |
| Publish Date |
2020-09-22 03:17 |
| Citation |
Ballotin VR, Bigarella LG, Riva F, Onzi G,Balbinot RA, Balbinot SS, Soldera J. Primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome associated with inflammatory bowel disease: A case report and systematic review. World J Clin Cases 2020; 8(18): 4075-4093 |
| URL |
https://www.wjgnet.com/2307-8960/full/v8/i18/4075.htm |
| DOI |
https://dx.doi.org/10.12998/wjcc.v8.i18.4075 |
