Immunological features of patients with primary biliary cirrhosis (PBC) overlapping systemic sclerosis: a comparison with patients with PBC alone

J Gastroenterol Hepatol. 1998 Sep;13(9):897-901. doi: 10.1111/j.1440-1746.1998.tb00758.x.


To characterize the immunological features of patients with primary biliary cirrhosis (PBC) overlapping systemic sclerosis (SSc), 26 patients with PBC were classified according to the presence of scleroderma-related features (Raynaud's phenomenon, sclerodactyly etc.). The patients were classified into 10 patients with PBC overlapping SSc (PBC-SSc), four patients with some scleroderma-related features although not meeting the criteria of SSc (PBC-SSc spectrum) and 12 patients with PBC alone. Sera from PBC-SSc showed a significantly higher positivity to anti-centromere antibody (P< 0.01) and to E1beta of pyruvate dehydrogenase complex (P< 0.005) than those from patients with PBC alone. The same tendency was observed in PBC-SSc spectrum patients. Patients with PBC exhibiting scleroderma-related features, in both the PBC-SSc and PBC-SSc spectrum, may comprise a subset in PBC, not only clinically, but also immunologically.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Aged
  • Autoantibodies / analysis*
  • Centromere / immunology*
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Humans
  • Liver Cirrhosis, Biliary / classification
  • Liver Cirrhosis, Biliary / immunology*
  • Male
  • Middle Aged
  • Mitochondria / immunology*
  • Pyruvate Dehydrogenase Complex / immunology*
  • Scleroderma, Systemic / immunology*


  • Autoantibodies
  • Pyruvate Dehydrogenase Complex