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

This article has now been updated. Please use the final version.

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

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Keywords: immunoglobulin G4-related autoimmune hepatitis, autoimmune pancreatitis, autoimmune hepatitis, immunoglobulin G4-related disease, immunoglobulin G4, steroid

JOURNAL OPEN ACCESS Advance online publication

Article ID: 4687-24

DOI https://doi.org/10.2169/internalmedicine.4687-24

The final version of this article is now available: Vol. 64 (2025), No. 11 pp. 1659-1665

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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.

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