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Immunoglobulin G4-related autoimmune hepatitis simultaneously concomitant with autoimmune pancreatitis: a case report

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Abstract

Thus far, there have been limited case reports on immunoglobulin G4-related autoimmune hepatitis (IgG4-AIH), and its clinical features have not been elucidated. We herein report a rare case of IgG4-AIH simultaneously concomitant with autoimmune pancreatitis (AIP). A 73-year-old female was admitted to our hospital for further investigation of elevated levels of liver transaminase and pancreatic enzymes. Her serological tests showed a high antinuclear antibody titer, and elevated IgG and IgG4 levels. Liver biopsy revealed interface hepatitis and bridging necrosis with IgG4-positive lymphoplasmacytic infiltration in the portal area. Moreover, contrast-enhanced computed tomography (CECT) showed pancreatic tail enlargement, and magnetic resonance cholangiopancreatography showed skipped narrowing of the main pancreatic duct in the pancreatic tail. Endoscopic ultrasonography-fine needle aspiration specimens showed no malignant cells. Based on these results, we diagnosed her with IgG4-AIH simultaneously concomitant with probable type 1 AIP. She was started on prednisolone (PSL) at 35 mg/d, and her symptoms and liver transaminase levels improved. One month after starting treatment, CECT showed improvement of pancreatic tail enlargement. She is maintained on 5 mg PSL/d and has been in remission for two years.

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Abbreviations

IgG4-RD:

Immunoglobulin G 4-related disease

AIP:

Autoimmune hepatitis

IgG4-AIH:

IgG4-related autoimmune hepatitis

CECT:

Contrast-enhanced computed tomography

MRCP:

Magnetic resonance cholangiopancreatography

EUS:

Endoscopic ultrasonography

EUS-FNA:

Endoscopic ultrasonography-fine needle aspiration

IHC:

Immunohistochemistry

HPF:

High-power field

IAIHG:

International autoimmune hepatitis group

PSL:

Prednisolone

UDCA:

Ursodeoxycholic acid

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Acknowledgements

This work was partially supported by the Japanese Society for the Promotion of Science (JSPS) KAKENHI grant number JP20K16962 (to YY).

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Authors and Affiliations

  1. Department of Gastroenterology, Otaru City General Hospital, Otaru, Japan

    Yoshihiro Yokoyama, Keiko Okuda, Hajime Sasaki, Ryo Suzuki, Takashi Yabana, Yoshiaki Arimura & Yoshihiro Kondo

  2. Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Chuo-ku Sapporo, Sapporo, Hokkaido, S1W16060-8543, Japan

    Yoshihiro Yokoyama, Noriyuki Akutsu, Yasunao Numata, Yoshiharu Masaki & Hiroshi Nakase

  3. Department of Clinical Pathology, Otaru City General Hospital, Otaru, Japan

    Rena Morita

Authors
  1. Yoshihiro Yokoyama
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  2. Noriyuki Akutsu
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  3. Yasunao Numata
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  4. Keiko Okuda
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  5. Rena Morita
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  9. Takashi Yabana
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  11. Yoshihiro Kondo
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  12. Hiroshi Nakase
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Correspondence to Yoshihiro Yokoyama.

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Yoshihiro Yokoyama, Noriyuki Akutsu, Yasunao Numata, Keiko Okuda, Rena Morita, Yoshiharu Masaki, Hajime Sasaki, Ryo Suzuki, Takashi Yabana, Yoshiaki Arimura, Yoshihiro Kondo and Hiroshi Nakase declare that they have no conflict of interest.

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All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Yokoyama, Y., Akutsu, N., Numata, Y. et al. Immunoglobulin G4-related autoimmune hepatitis simultaneously concomitant with autoimmune pancreatitis: a case report. Clin J Gastroenterol 14, 1740–1745 (2021). https://doi.org/10.1007/s12328-021-01509-0

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  • DOI: https://doi.org/10.1007/s12328-021-01509-0

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