Jarasvaraparn C, Imran H, Siddiqui A, Wilson F, Gremse DA. Association of autoimmune hepatitis type 1 in a child with Evans syndrome. World J Hepatol 2017; 9(23): 1008-1012 [PMID: 28878866 DOI: 10.4254/wjh.v9.i23.1008]
Corresponding Author of This Article
David A Gremse, MD, FAAP, FACG, Department of Pediatrics, University of South Alabama, 1601 Center St, Mobile, AL 36604, United States. dgremse@health.southalabama.edu
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
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 Hepatol. Aug 18, 2017; 9(23): 1008-1012 Published online Aug 18, 2017. doi: 10.4254/wjh.v9.i23.1008
Association of autoimmune hepatitis type 1 in a child with Evans syndrome
Chaowapong Jarasvaraparn, Hamayun Imran, Abdul Siddiqui, Felicia Wilson, David A Gremse
Chaowapong Jarasvaraparn, Hamayun Imran, Abdul Siddiqui, Felicia Wilson, Department of Pediatrics, University of South Alabama, Mobile, AL 36604, United States
David A Gremse, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of South Alabama, Mobile, AL 36604, United States
Author contributions: All authors contributed to the acquisition of data, writing, and revision of this manuscript.
Institutional review board statement: This case report was exempt from the Institutional Review Board standards at University of South Alabama.
Informed consent statement: Our patient’s legal guardian provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors have no conflicts of interest to disclose.
Open-Access: 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/
Correspondence to: David A Gremse, MD, FAAP, FACG, Department of Pediatrics, University of South Alabama, 1601 Center St, Mobile, AL 36604, United States. dgremse@health.southalabama.edu
Telephone: +1-251-4343919
Received: February 10, 2017 Peer-review started: February 15, 2017 First decision: April 17, 2017 Revised: June 27, 2017 Accepted: July 7, 2017 Article in press: July 10, 2017 Published online: August 18, 2017
Abstract
Autoimmune hepatitis (AIH) is a progressive liver disease that is often associated with extrahepatic autoimmune disorders. Evans syndrome (ES) is a rare autoimmune disorder, which is characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Association of AIH with ES is rare, especially in children. We report a 3-year-old female with a past medical history of ES who presented with jaundice and significant transaminitis due to AIH type 1. She required multiple treatments with steroids as well as azathioprine, intravenous immunoglobulin and a course of rituximab.
Core tip: We report a 3-year-old female with a past medical history of Evans syndrome (ES) who presented with jaundice and significant transaminitis due to autoimmune hepatitis (AIH) type 1. To our knowledge, this is a rare association of concurrent AIH and ES in a child who responded well to rituximab. The patient also demonstrated short-term response to intravenous immunoglobulin, methylprednisolone, azathioprine and oral prednisone. We conclude that ES may evolve over a period of several months therefore evaluation for associated autoimmune conditions should be considered in these patients.
