Published online Sep 6, 2020. doi: 10.12998/wjcc.v8.i17.3828
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
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.
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.
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.