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 [PMID: 33024765 DOI: 10.12998/wjcc.v8.i18.4075]
Corresponding Author of This Article
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
Research Domain of This Article
Medicine, Research & Experimental
Article-Type of This Article
Systematic Reviews
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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/
World J Clin Cases. Sep 26, 2020; 8(18): 4075-4093 Published online Sep 26, 2020. doi: 10.12998/wjcc.v8.i18.4075
Primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome associated with inflammatory bowel disease: A case report and systematic review
Vinícius Remus Ballotin, Lucas Goldmann Bigarella, Floriano Riva, Georgia Onzi, Raul Angelo Balbinot, Silvana Sartori Balbinot, Jonathan Soldera
Vinícius Remus Ballotin, Lucas Goldmann Bigarella, Faculty of Medicine, Universidade de Caxias do Sul (UCS), Caxias do Sul 95070560, Brazil
Floriano Riva, CPM Laboratório de Caxias do Sul, Caxias do Sul 95070561, Brazil
Georgia Onzi, Raul Angelo Balbinot, Silvana Sartori Balbinot, Jonathan Soldera, Clinical Gastroenterology, Universidade de Caxias do Sul (UCS), Caxias do Sul 95070560, Brazil
Author contributions: Ballotin VR, Bigarella LG, Riva F, Onzi G, Balbinot RA, Balbinot SS, Soldera J participated in the concept and design of the research, drafted the manuscript and contributed to data acquisition, analysis and interpretation; Ballotin VR contributed to statistical analysis; Soldera J contributed to study supervision; all authors contributed to critical revision of the manuscript for important intellectual content.
Conflict-of-interest statement: All the authors declare that they have no competing interests.
PRISMA 2009 Checklist statement: The guidelines of the PRISMA 2009 statement have been adopted.
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/
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
Received: March 21, 2020 Peer-review started: March 21, 2020 First decision: April 8, 2020 Revised: April 23, 2020 Accepted: July 30, 2020 Article in press: July 30, 2020 Published online: September 26, 2020
Core Tip
Core Tip: We report the case of a female patient with autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome associated with ulcerative colitis and 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 associated dilations. Liver biopsy demonstrated periportal necroinflammatory activity, plasmocyte infiltration, and advanced fibrosis. Colonoscopy showed ulcerative pancolitis and mild activity (Mayo score 1), with a spared rectum. Treatment with corticosteroids, azathioprine, ursodeoxycholic acid and mesalamine was initiated, with improvement in laboratory tests. Searches for systematic reviews 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. Of 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 has low mortality and good response to treatment.
