Tan YW, Wang JM, Chen L. Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report. World J Clin Cases 2021; 9(25): 7527-7534 [PMID: 34616822 DOI: 10.12998/wjcc.v9.i25.7527]
Corresponding Author of This Article
You-Wen Tan, MD, Chief Doctor, Professor, Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, No. 300 Danjiamen, Runzhou District, Zhenjiang 212003, Jiangsu Province, China. tyw915@sina.com
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 Clin Cases. Sep 6, 2021; 9(25): 7527-7534 Published online Sep 6, 2021. doi: 10.12998/wjcc.v9.i25.7527
Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report
You-Wen Tan, Jia-Min Wang, Li Chen
You-Wen Tan, Jia-Min Wang, Li Chen, Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, Zhenjiang 212003, Jiangsu Province, China
Author contributions: Tan YW and Wang JM contributed equally to this work; Chen L collected and analyzed the data, and drafted the manuscript; Tan YW and Wang JM wrote and revised the manuscript; all authors have read and approved the final version to be published.
Informed consent statement: Informed consent was obtained from the patient for publication of this case report.
Conflict-of-interest statement: All authors have no conflict of interest related to the manuscript.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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: You-Wen Tan, MD, Chief Doctor, Professor, Department of Hepatology, The Third Hospital of Zhenjiang Affiliated Jiangsu University, No. 300 Danjiamen, Runzhou District, Zhenjiang 212003, Jiangsu Province, China. tyw915@sina.com
Received: February 18, 2021 Peer-review started: February 18, 2021 First decision: April 18, 2021 Revised: April 24, 2021 Accepted: July 19, 2021 Article in press: July 19, 2021 Published online: September 6, 2021
Abstract
BACKGROUND
The immune-mediated invasion of IgG4-positive plasma cells in the liver is found in some autoimmune hepatitis. Giant-cell hepatitis (GCH) is a very rare pathological feature in adults, and the clinical characteristics of the simultaneous appearance of the two pathological phenomena are not clear.
CASE SUMMARY
A 68-year-old woman was hospitalized with fatigue, poor appetite, and yellow urine for 20 d. Liver function tests and immunological indexes were significantly abnormal and accompanied by elevated serum IgG4 levels. Liver pathology revealed severe inflammation of the interface between the portal tract and hepatocytes, portal area inflammation, plasma cell infiltration, formation of rosette cells, IgG4-positive plasma cells > 10/high-power field, IgG4/IgG > 40%, and multinucleated liver cell swelling. IgG4-related autoimmune hepatitis (AIH) combined with GCH was diagnosed, and methylprednisolone was administered at 40 mg/day. Two weeks later, the clinical symptoms disappeared, and the liver function and immunological indicators were significantly improved. Methylprednisolone was reduced at a rate of 4–8 mg per week to 8 mg/day for maintenance. A second liver biopsy 48 wk later indicated that liver inflammation and fibrosis were significantly improved. IgG4-positive plasma cells and GCH were not detected. A literature search was conducted to analyze articles reporting similar pathological phenomena.
CONCLUSION
AIH with simultaneous IgG4-positive plasma cell infiltration and GCH, liver inflammation, and fibrosis is possibly more severe than typical AIH but sensitive to corticosteroids.
Core Tip: A 68-year-old woman was hospitalized for liver function tests. Her immunological indices differed significantly from normal, and were accompanied by elevated serum IgG4 levels. Liver pathology revealed severe inflammation at the interface between the portal tract and hepatocytes, plasma cell infiltration, formation of rosette cells, IgG4-positive plasma cells > 10/high-power field, IgG4/IgG > 40%, and multinucleated liver cell swelling. The patient was diagnosed as having IgG4-autoimmune hepatitis combined with giant-cell hepatitis (GCH), and 40 mg/day methylprednisolone was administered. A second liver biopsy 48 wk later indicated significant amelioration of liver inflammation and fibrosis. IgG4-positive plasma cells and GCHs were not detected. A literature search was conducted to analyze reports of similar pathological phenomena.
