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

著者情報

キーワード: autoimmune liver disease, immunoglobulin G4-related sclerosing cholangitis, overlap, primary biliary cirrhosis

ジャーナルオープンアクセス

2014 年 53 巻 13 号 p. 1429-1433

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

詳細

抄録

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.

責任著者(Corresponding author)

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