A case of primary biliary cirrhosis accompanied with fibrinogen storage disease

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

研究成果: Article査読

7 被引用数 (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.

ジャーナルPathology Research and Practice
出版ステータスPublished - 2005 5月 23

ASJC Scopus subject areas

  • 病理学および法医学
  • 細胞生物学


「A case of primary biliary cirrhosis accompanied with fibrinogen storage disease」の研究トピックを掘り下げます。これらがまとまってユニークなフィンガープリントを構成します。