Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

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 a...

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Veröffentlicht in:Internal Medicine 2014, Vol.53(13), pp.1429-1433
Hauptverfasser: Takemoto, Reika, Miyake, Yasuhiro, Harada, Kenichi, Nakanuma, Yasuni, Moriya, Akio, Ando, Masaharu, Hirohata, Mamoru, Yamamoto, Kazuhide
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container_end_page 1433
container_issue 13
container_start_page 1429
container_title Internal Medicine
container_volume 53
creator Takemoto, Reika
Miyake, Yasuhiro
Harada, Kenichi
Nakanuma, Yasuni
Moriya, Akio
Ando, Masaharu
Hirohata, Mamoru
Yamamoto, Kazuhide
description 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.
doi_str_mv 10.2169/internalmedicine.53.2250
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subjects autoimmune liver disease
Bezafibrate - therapeutic use
Cholagogues and Choleretics - therapeutic use
Cholangitis, Sclerosing - complications
Cholangitis, Sclerosing - diagnosis
Drug Therapy, Combination
Humans
Immunoglobulin G - blood
immunoglobulin G4-related sclerosing cholangitis
Liver Cirrhosis, Biliary - complications
Liver Cirrhosis, Biliary - diagnosis
Male
Middle Aged
overlap
primary biliary cirrhosis
Ursodeoxycholic Acid - therapeutic use
title Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis
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