Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

Reika Takemoto; Yasuhiro Miyake; Kenichi Harada; Yasuni Nakanuma; Akio Moriya; Masaharu Ando; Mamoru Hirohata; Kazuhide Yamamoto

doi:10.2169/internalmedicine.53.2250

CASE REPORTS

Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

Reika Takemoto, Yasuhiro Miyake, Kenichi Harada, Yasuni Nakanuma, Akio Moriya, Masaharu Ando, Mamoru Hirohata, Kazuhide Yamamoto

Author information

Reika Takemoto
Department of Internal Medicine, Mitoyo General Hospital, Japan
Yasuhiro Miyake
Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan
Kenichi Harada
Department of Human Pathology, Kanazawa University Graduate School of Medicine, Japan
Yasuni Nakanuma
Department of Human Pathology, Kanazawa University Graduate School of Medicine, Japan
Akio Moriya
Department of Internal Medicine, Mitoyo General Hospital, Japan
Masaharu Ando
Department of Internal Medicine, Mitoyo General Hospital, Japan
Mamoru Hirohata
Department of Internal Medicine, Mitoyo General Hospital, Japan
Kazuhide Yamamoto
Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan

Keywords: autoimmune liver disease, immunoglobulin G4-related sclerosing cholangitis, overlap, primary biliary cirrhosis

JOURNAL OPEN ACCESS

2014 Volume 53 Issue 13 Pages 1429-1433

DOI https://doi.org/10.2169/internalmedicine.53.2250

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Abstract

We herein present a case of an overlap of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) and primary biliary cirrhosis (PBC). A 61-year-old man was diagnosed with PBC due to abnormal liver biochemical tests and positivity for serum anti-M2 antibody. However, his response to bezafibrate and ursodeoxycholic acid was insufficient. Five years later, his serum IgG4 level was found to increase. His liver biopsy specimens showed features of nonsuppurative destructive cholangitis in some portal tracts and periductal fibrosis with dense infiltration of IgG4-positive cells in other portal tracts. This case demonstrates that the serum IgG4 level may be worth measuring in patients with PBC refractory to conventional treatment.

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