Clinical and immunological characterization of patients with systemic sclerosis overlapping primary biliary cirrhosis: a comparison with patients with systemic sclerosis alone

J Dermatol. 1999 Jan;26(1):18-22. doi: 10.1111/j.1346-8138.1999.tb03503.x.


Limited cutaneous systemic sclerosis (lSSc) or CREST syndrome is sometimes complicated by primary biliary cirrhosis (PBC). To characterize the clinical and immunological features of patients with SSc overlapping PBC (SSc-PBC), the clinical and laboratory data of 11 SSc-PBC were studied. Since all of the SSc-PBC were lSSc, the features of SSc-PBC were compared with those of 266 patients with lSSc alone. The sera from SSc-PBC showed a significantly higher positivity for anti-centromere antibody (ACA) (p < 0.0005) and higher reactivity to centromere protein-C. Although SSc-PBC more frequently exhibited calcinosis and telangiectasia (p < 0.05), there was no statistical difference in other features of CREST syndrome. There was no statistical difference in other scleroderma-related features either. SSc-PBC were more frequently complicated with Sjögren syndrome (p < 0.05). The most prominent feature of SSc-PBC was a higher incidence of ACA than in lSSc alone.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Aged
  • Autoantibodies / analysis
  • Blotting, Western
  • CREST Syndrome / complications
  • CREST Syndrome / immunology
  • CREST Syndrome / pathology
  • Centromere / immunology
  • Humans
  • Liver Cirrhosis, Biliary / complications*
  • Middle Aged
  • Mitochondria / immunology
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / immunology*
  • Scleroderma, Systemic / pathology*


  • Autoantibodies