A case of autoimmune hepatitis/primary biliary cirrhosis overlap syndrome progressing rapidly to cirrhosis with skin pigmentation and various immunological characteristics

Kazufumi Dohmen, Hirofumi Tanaka, MasatoraHaruno, Shin Ichi Aishima, Shinji Shimoda

Research output: Contribution to journalArticle

Abstract

A 53-year-old Japanese man was found to have a liver dysfunction with a total bilirubin of 3.3 mg/dl, ALT 724 IU/l, alkaline phosphatase 568IU/l with an elevation of serum IgM while serum levels of IgG, antinuclear antibody (ANA) and anti-mitochondrial antibody (AMA) showed normal. Primary biliary cirrhosis (PBC) was suspected at that time, however, he left the hospital by self-determination. Five years later, he developed liver cirrhosis with jaundice, ascites and skin pigmentation and sclerosis. Antinuclear antibody, negative 5 years before, was now positive at 1: more than 2560. Serum IgG and IgM were elevated. Anti-mitochondrial antibody M2 component, and liver-kidney microsome (LKM)-1 antibody, double strand DNA antibody, platelet-associated IgG, direct-, indirect Coombs tests were also positive. Liver histology revealed cirrhosis. He was diagnosed as having AIH/PBC overlap syndrome due to serologic features. Sequential changes of the titers of several antibodies, laboratory characteristics and clinical symptoms within short durations are discussed.

Original languageEnglish
Pages (from-to)513-520
Number of pages8
JournalActa Hepatologica Japonica
Volume51
Issue number9
DOIs
Publication statusPublished - Nov 8 2010

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Skin Pigmentation
Autoimmune Hepatitis
Biliary Liver Cirrhosis
Coombs Test
Fibrosis
Immunoglobulin G
Antinuclear Antibodies
Immunoglobulin M
Antibodies
Anti-Idiotypic Antibodies
Insemination, Artificial, Homologous
Serum
Personal Autonomy
Liver Microsomes
Sclerosis
Jaundice
Bilirubin
Ascites
Liver Cirrhosis
Alkaline Phosphatase

All Science Journal Classification (ASJC) codes

  • Hepatology

Cite this

A case of autoimmune hepatitis/primary biliary cirrhosis overlap syndrome progressing rapidly to cirrhosis with skin pigmentation and various immunological characteristics. / Dohmen, Kazufumi; Tanaka, Hirofumi; MasatoraHaruno; Aishima, Shin Ichi; Shimoda, Shinji.

In: Acta Hepatologica Japonica, Vol. 51, No. 9, 08.11.2010, p. 513-520.

Research output: Contribution to journalArticle

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