A case of primary biliary cirrhosis accompanied with fibrinogen storage disease

Hiroko Mitsui, Eisaku Miyauchi, Jun Miyahara, Kiriko Wada, Mitsunori Yamakawa, Sumio Kawata

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10 Citations (Scopus)


We report the first case of primary biliary cirrhosis (PBC) accompanied by fibrinogen storage disease (FSD). A 50-year-old Japanese woman had been treated for numbness of her right-side extremities for 5 years. Mildly elevated serum levels of alkaline phosphatase and γ-glutamyl transferase were detected. The titers of both anti-mitochondrial (× 320) and anti-mitochondrial M2 (× 84) antibodies were elevated. The biopsied liver specimen showed mononuclear cell infiltrate densely encircling the bile ducts, poorly developed epithelioid cell granuloma, and loss of integrity of bile duct organization, which permitted a diagnosis of stage 1 PBC according to Scheuer's histologic classification. In addition, round to oval, eosinophilic, homogenous intracytoplasmic inclusions, several μm in average size, with a surrounding halo were found in the vast majority of hepatocytes. These inclusions were negative for the periodic acid-Schiff reaction. In immunohistochemistry, the inclusions were positive for fibrinogen and complement C3c, but not for HBs antigen and α1-antitrypsin. These findings were identical to FSD. To investigate the mechanism(s) of abnormal fibrinogen storage, immunostaining for heat shock protein 70 and ubiquitin was performed. The former was detected in all intracytoplasmic inclusions, whereas the latter was detected in only some inclusions, suggesting a partial loss of ubiquitin expression.

Original languageEnglish
Pages (from-to)341-345
Number of pages5
JournalPathology Research and Practice
Issue number4
Publication statusPublished - 2005 May 23


  • Fibrinogen storage disease
  • Heat shock protein 70
  • Primary biliary cirrhosis
  • Ubiquitin


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