ABSTRACT
BACKGROUND: A specific antinuclear antibody for primary biliary cholangitis (PBC) is anti-Sp100, which was recognized as a serological marker of concurrent urinary tract infection. We sought to determine the clinical characteristics of PBC patients who had anti-Sp100. PATIENTS AND METHODS: Fifty-one patients with PBC and 10 healthy controls (HCs) were enrolled. Anti-Sp100 were determined with an ELISA method. Lipopolysaccharide-binding protein (LBP) was measured as a serological hallmark for bacterial infection. The correlations of anti-Sp100 with demographic, laboratory, and pathological parameters were investigated. RESULTS: Six of the 51 (11.8%) PBC patients had anti-Sp100, whereas none of the HCs did. There was no significant difference in the frequency of antimitochondrial antibodies (AMAs) between PBC patients with and without anti-Sp100 (67% vs. 82%, p = 0.5839). Biochemical and immunological parameters were not associated with the emergence of anti-Sp100 in these patients. The clinical stage by Scheuer classification was not correlated with the existence of anti-Sp100. No significant difference in the serum LBP levels was found between PBC patients with and without anti-Sp-100, although serum LBP levels were significantly higher in PBC patients with anti-Sp100 than in HCs (8.30 ± 2.24 ng/ml, vs. 5.12 ± 2.48 ng/ml, p = 0.0022). The frequency of granuloma formation was higher in the liver specimens of PBC patients with anti-Sp100 than in those without anti-Sp100 (67% vs 29%, p = 0.0710). CONCLUSION: anti-Sp100 does not become a complementary serological marker for PBC in AMA-negative patients. A bacterial infection may trigger the production of anti-Sp100. Another factor is required to initiate the autoantibody production.