High prevalence of primary biliary cirrhosis and disease-associated autoantibodies in Japanese patients with systemic sclerosis
Sayako Imura-Kumada1 , Minoru Hasegawa1 , Takashi Matsushita1 , Yasuhito Hamaguchi1 , Susan Encabo2 , Zakera Shums2 , Gary L. Norman2 , Kazuhiko Takehara1 , Manabu Fujimoto1
11 September 2011
25 January 2012
12 February 2012
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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 (antigp210) 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 antigp210 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.
Systemic sclerosis - Primary biliary cirrhosis - Anti-mitochondrial antibodies - Anti-sp100 antibodies - Anti-gp210 antibodies