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©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
Autoimmune hepatitis in a patient with immunoglobulin A nephropathy: A case report
You Hyun Jeon, Da Woon Kim, So Jeong Lee, Young Joo Park, Hyo Jin Kim, Miyeun Han, Il Young Kim, Dong Won Lee, Sang Heon Song, Soo Bong Lee, Eun Young Seong
You Hyun Jeon, Da Woon Kim, Department of Internal Medicine, Pusan National University School of Medicine, Busan 49241, South Korea
So Jeong Lee, Department of Pathology, Pusan National University Hospital, Busan 49241, South Korea
Young Joo Park, Division of Gastroenterology, Department of Internal Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
Hyo Jin Kim, Miyeun Han, Sang Heon Song, Eun Young Seong, Division of Nephrology, Department of Internal Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, South Korea
Il Young Kim, Dong Won Lee, Soo Bong Lee, Division of Nephrology, Department of Internal Medicine, Pusan National University Yangsan Hospital, Yangsan 626-770, South Korea
Author contributions: Jeon YH collected clinical data and contributed manuscript drafting; Kim DW collected clinical data and drafted table and figures; Lee SJ performed the histological analyses and interpretation; Park YJ was physician who provided treatment and intellectual content; Kim HJ, Han M, Kim IY and Lee DW reviewed the manuscript and provided intellectual content; Song SH, Lee SB and Seong EY reviewed the literature and revised the manuscript; all authors approved the final version of the manuscript.
Supported by Pusan National University Hospital Education and Research Team, No 219.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
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: Eun Young Seong, MD, PhD, Associate Professor, Division of Nephrology, Department of Internal Medicine, Biomedical Research Institute, Pusan National University Hospital, 179 Gudeok-ro, Seo-gu, Busan 49241, South Korea.
sey-0220@hanmail.net
Received: June 29, 2020
Peer-review started: June 29, 2020
First decision: July 24, 2020
Revised: August 5, 2020
Accepted: August 20, 2020
Article in press: August 20, 2020
Published online: September 6, 2020
Processing time: 66 Days and 22.2 Hours
BACKGROUND
Immunoglobulin A nephropathy (IgAN) is the most commonly encountered glomerular disease in Asian countries. It has a broad clinical presentation, and it is frequently associated with other conditions. Chronic liver disease is well recognized as the leading cause of secondary IgAN. However, cases of IgAN associated with autoimmune hepatitis (AIH) have seldom been reported.
CASE SUMMARY
A 63-year-old Korean woman was admitted to Pusan National University Hospital for an evaluation of abdominal pain and elevated liver enzymes. Two weeks prior, she had presented to our hospital with proteinuria of approximately 1350 mg/d and hematuria and was diagnosed with IgAN. Autoimmune profiles were highly positive for antinuclear antibodies, and symptoms related to portal hypertension including ascites and peripheral edema were present. A diagnosis of AIH was made according to the simplified scoring system of the International Autoimmune Hepatitis Group. Despite immunosuppression with prednisolone and azathioprine, rapid deterioration of liver function led to end-stage liver disease. After a living-donor liver transplantation, liver function gradually improved, and she had maintained stable liver and kidney function at the six months follow-up.
CONCLUSION
Cases of secondary IgAN with chronic liver disease have been frequently reported in the literature but are rarely associated with AIH. We encountered an IgAN patient with concurrent progressive liver failure due to AIH.
Core tip: Immunoglobulin A nephropathy (IgAN) is an autoimmune disease and may be related to other autoimmune conditions. To the best of our knowledge, only two cases of IgAN with autoimmune hepatitis have been reported, and the pathophysiological associations of both diseases have not been established.
