[Case of primary biliary cirrhosis patient with anti-p97/VCP antibodies presenting a mild clinical course].

In this study, we present a case of mild PBC that had anti-p97/VCP. A 53-year-old woman had been suspected of having chronic liver disease since 1983. In 1998, she visited the clinic, complaining of struma and pruritus. Laboratory findings on the first visit showed elevated levels of alkaline phosphatase (ALP) 454 IU/l(110-360), gammaGTP 250IU/l(-45) and IgM 671mg/dl(35-220). A screening of anti-mitochondrial antibody test was positive at a 1:80 dilution. A liver biopsy specimen revealed PBC at Scheuer stage 1. Following a treatment of ursodeoxycolic acid (UDCA) 300mg/day for 6 months, AMA and IgM were reduced to 1:20 and 220mg/dl, respectively. However, she was found to have low titer of anti-p97/VCP antibodies, determined by immunoprecipitation of radiolabeled recombinant protein produced by in vitro translation and transcription of the full length p97 cDNA. She has continued to be clinically stable following administration of UDCA 300mg. A PBC patient with anti-p97/VCP antibody showed a milder clinical course, suggesting some beneficial role of this antibody.

Prevalence of antimitochondrial antibody in Japanese corporate workers in Kanagawa prefecture.

BACKGROUND: The prevalence of antimitochondrial antibody (AMA) in humans and its relationship to the development of primary biliary cirrhosis (PBC) are not well known. We have estimated the frequency of AMA in the general population, and studied its association with PBC. METHODS: We studies 1714 corporate workers (median age, 48 years; range, 30 to 59 years) who had an annual health check from 1998 to 1999 at Kawasaki Social Insurance Hospital in Japan. We used an indirect immunofluorescence method for screening serum AMA. We applied the prevalence of AMA-positive people in the study group to the general population in Japan. Then the inferred AMA-positive population was compared to the actual number of patients with PBC in statistics published by the Japanese Government. RESULTS: AMA was detected in 11 of 1714 people (0.64%; 95% confidence interval, 0.26% to 1.02%). All these 11 sera reacted with 2-oxoacid-dehydrogenase complex by immunoblotting. Of these 11 individuals, none had subjective symptoms, all had normal serum bilirubin levels, and 6 had abnormal liver function test results. Using published statistics for the Japanese population, we inferred that there were approximately 336,472 AMA-positive people in Japan from age 30 to 59 years. The number of patients with symptomatic PBC recorded by the nationwide epidemiological survey of the Japanese Government was 2459. Thus, we inferred the rate of symptomatic PBC among AMA-positive persons to be about 0.73% (2459/336,472). CONCLUSIONS: AMA is not a rare antibody in the general population, but few people develop recognizable PBC even if they have AMA.

Profile and clinical significance of anti-nuclear envelope antibodies found in patients with primary biliary cirrhosis: a multicenter study.

Primary biliary cirrhosis (PBC) sera contain antibodies which recognize various nuclear envelope proteins of which antibody against gp210 has been proven to be diagnostic for disease. In contrast, the clinical significance of another nuclear envelope antibody, anti-p62 antibody has not been well investigated. In the present study, we have analyzed anti-nuclear envelope antibodies by indirect immunofluorescence and immunoblot using rat liver nuclear envelope proteins and wheat germ agglutinin-bound fraction. Test sera were obtained from 175 patients with PBC and from 120 controls. Anti-gp210, anti-lamina associated polypeptide 2, anti-lamin B receptor, and anti-p62 complex antibodies were detected with a frequency of 26% (46 of 175), 6% (11 of 175), 9% (16 of 175), and 13% (15 of 115), respectively. The confirmation of Scheuer's stage IV was made with a frequency of 27% (4 of 15) in PBC patients with anti-p62 complex antibody, in contrast to only 2% (2 of 100) in PBC patients without anti-p62 complex antibody. This difference was found to be statistically significant. The presence of anti-p62 complex antibody may be related with the progressive or advanced state of PBC.