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A case of primary biliary cholangitis overlapping with type 2 autoimmune hepatitis

  • Nozomi Amano
  • Sho Sato
  • Ayato Murata
  • Hironori Tsuzura
  • Ko Tomishima
  • Shunsuke Sato
  • Kohei Matsumoto
  • Yuji Shimada
  • Katsuyori Iijima
  • Kenichi Harada
  • Takuya GendaEmail author
Case Report
  • 25 Downloads

Abstract

A 42-year-old woman was admitted to our hospital with cholestatic liver injury. Serological examination revealed anti-mitochondrial M2 antibody positivity and anti-nuclear antibody and anti-smooth muscle antibody negativity. Histological examination of the first liver biopsy revealed chronic nonsuppurative destructive cholangitis with epithelioid granulomas. Ursodeoxycholic acid therapy successfully treated her cholestasis. Sixteen months later, she developed acute icteric hepatitis with elevation of serum aspartate and alanine aminotransferase levels. Anti-mitochondrial M2 positivity and anti-nuclear antibody and anti-smooth muscle antibody negativity persisted at that time. However, it became clear that anti-liver kidney microsomal type 1 antibody was positive. Histological examination of the second liver biopsy demonstrated scarce interface hepatitis and evident parenchymal inflammation and centrilobular zonal necrosis. Her liver biochemical test results promptly improved with the addition of prednisolone therapy. Considering the findings, she was diagnosed with primary biliary cholangitis–type 2 autoimmune hepatitis overlap syndrome. According to a literature review, this is an extremely rare autoimmune overlap syndrome.

Keywords

Anti-mitochondrial antibody Anti-liver kidney microsomal antibody Autoimmune hepatitis Chronic nonsuppurative destructive cholangitis Primary biliary cholangitis 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interests.

Human and animal rights

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

Informed consent

Informed consent was obtained from the patient in the report.

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Copyright information

© Japanese Society of Gastroenterology 2019

Authors and Affiliations

  • Nozomi Amano
    • 1
  • Sho Sato
    • 1
  • Ayato Murata
    • 1
  • Hironori Tsuzura
    • 1
  • Ko Tomishima
    • 1
  • Shunsuke Sato
    • 1
  • Kohei Matsumoto
    • 1
  • Yuji Shimada
    • 1
  • Katsuyori Iijima
    • 1
  • Kenichi Harada
    • 2
  • Takuya Genda
    • 1
    Email author
  1. 1.Department of Gastroenterology and HepatologyJuntendo University Shizuoka HospitalIzunokuniJapan
  2. 2.Department of Human PathologyKanazawa University Graduate School of MedicineKanazawaJapan

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