Immunoglobulin G4-related autoimmune hepatitis following type 1 autoimmune pancreatitis: A case report and literature review

Chiharu Toh; Shinichi Morita; Nobutaka Takeda; Fusako Yamazaki; Kunihiko Yokoyama; Masatoshi Sato; Daisuke Kumaki; Takeshi Sakai; Kazuhiro Funakoshi; Koichi Tsuneyama

doi:10.2169/internalmedicine.4687-24

Immunoglobulin G4-related autoimmune hepatitis following type 1 autoimmune pancreatitis: A case report and literature review

Intern Med. 2024 Nov 8. doi: 10.2169/internalmedicine.4687-24. Online ahead of print.

Affiliations

¹ Department of Gastroenterology and Hepatology, Niigata Prefectural Central Hospital, Japan.
² Department of Pathology, Niigata Prefectural Central Hospital, Japan.
³ Department of Pathology and Laboratory Medicine, Tokushima University Graduate School of Biomedical Science, Japan.

PMID: 39522990
DOI: 10.2169/internalmedicine.4687-24

Abstract

We herein report a case of IgG4-related autoimmune hepatitis (AIH) in a patient with a history of type 1 autoimmune pancreatitis. A 56-year-old man presented with fatigue and jaundice at our hospital. A blood biochemistry analysis revealed significant liver dysfunction, positive results for antinuclear antibodies, and high serum IgG4 levels. A histopathological examination revealed interface hepatitis marked by IgG4-positive plasma cell infiltration in the portal area, leading to liver cell depletion and necrosis. Based on the diagnosis of IgG4-related AIH, prednisolone treatment was initiated, which led to the rapid resolution of liver dysfunction and jaundice. An accurate diagnosis of IgG4-related AIH is crucial to prevent secondary manifestations.

Keywords: autoimmune hepatitis; autoimmune pancreatitis; immunoglobulin G4; immunoglobulin G4-related autoimmune hepatitis; immunoglobulin G4-related disease; steroid.