High prevalence of primary biliary cirrhosis and disease-associated autoantibodies in Japanese patients with systemic sclerosis.
OBJECTIVE
To investigate the prevalence of primary biliary cirrhosis (PBC) and PBC-associated autoantibodies in Japanese systemic sclerosis (SSc) patients.


METHODS
Clinical data from 225 Japanese SSc patients were retrospectively obtained. Serum samples from these patients were examined for PBC-associated autoantibodies, anti-mitochondrial M2 antibodies (AMA), anti-sp100 antibodies (anti-sp100), and anti-gp210 antibodies (anti-gp210) by enzyme-linked immunosorbent assay.


RESULTS
Of 225 patients, 37 (16.4%) had AMA, 13 (5.8%) had anti-sp100, and 3 (1.3%) had anti-gp210. Three patients were positive for both AMA and anti-sp100, and 2 were positive for both AMA and anti-gp210. PBC was found in 22 (9.8%) patients positive for AMA with or without anti-sp100 or anti-gp210, but not in those with anti-sp100 or anti-gp210 without AMA. Furthermore, 13 patients lacking these three antibodies were diagnosed with or suspected of PBC by liver biopsy and/or their clinical manifestation. Multivariable analysis revealed that AMA and anti-centromere antibodies were independently associated with PBC in SSc patients, while anti-sp100 and anti-gp210 were not.


CONCLUSIONS
This study has demonstrated even higher prevalence of both PBC-associated autoantibodies and PBC in the Japanese SSc population than in the Caucasian SSc population. AMA and anti-centromere antibodies are likely to indicate increasing risk of PBC in SSc patients.
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan.
Sayako
S
Imura-Kumada
Minoru
M
Hasegawa
Takashi
T
Matsushita
Yasuhito
Y
Hamaguchi
Susan
S
Encabo
Zakera
Z
Shums
Gary L
GL
Norman
Kazuhiko
K
Takehara
Manabu
M
Fujimoto
D000328:Adult; D000368:Aged; D034961:Antigens, Nuclear / Q000276:immunology; D044466:Asian People; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D015897:Comorbidity; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000453:epidemiology* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D028861:Nuclear Pore Complex Proteins / Q000276:immunology; D015995:Prevalence; D012595:Scleroderma, Systemic / Q000097:blood / Q000453:epidemiology* / Q000276:immunology
D034961:Antigens, Nuclear; D001323:Autoantibodies; D001324:Autoantigens; C063865:NUP210 protein, human; D028861:Nuclear Pore Complex Proteins; C066642:SP100 protein, human