title: Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis.
abstract: OBJECTIVE
  To evaluate the prognosis of primary biliary cirrhosis (PBC) together with systemic sclerosis (SSc), as this is unknown.
  
  
  RESULTS
  A PBC database of 580 patients identified 43 with PBC and SSc: two patients with PBC alone were matched to each PBC-SSc patient for serum bilirubin concentration at the initial visit. Forty (93%) patients had limited cutaneous SSc. At diagnosis of PBC, median values were: 49.7 years, bilirubin 17 micromol/l, and albumin 40.5 g/l. Liver diagnosis occurred a median 4.9 years after SSc in 24 (56%) patients. In matched patients, median values at diagnosis were: 53.2 years, bilirubin 12 micromol/l, and albumin 41 g/l. Median follow up was similar: 3.16 years (PBC-SSc) and 4.8 years (PBC alone). The risk of transplantation or death from diagnosis, adjusting for sex, age, log bilirubin, and alkaline phosphatase was significantly lower in PBC-SSc (hazard ratio 0.116, p=0.01) due to less transplantation (hazard ratio 0.068, p=0.006). The rate of bilirubin increase was less in PBC-SSc (p=0.04). Overall survival was similar (hazard ratio 1.11, p=0.948); there were nine deaths (21%) in PBC-SSc (six SSc related and two liver related) and nine (11%) in PBC alone (six liver related).
  
  
  CONCLUSIONS
  Liver disease has a slower progression in PBC-SSc compared with matched patients with PBC alone.
authors.affiliation: Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, Pond St, Hampstead, London NW3 2QG, UK.
authors.forename: C
authors.initials: C
authors.lastname: Rigamonti
authors.forename: L M
authors.initials: LM
authors.lastname: Shand
authors.forename: M
authors.initials: M
authors.lastname: Feudjo
authors.forename: C C
authors.initials: CC
authors.lastname: Bunn
authors.forename: C M
authors.initials: CM
authors.lastname: Black
authors.forename: C P
authors.initials: CP
authors.lastname: Denton
authors.forename: A K
authors.initials: AK
authors.lastname: Burroughs
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000097:blood; D001663:Bilirubin / Q000097:blood; D002503:Centromere / Q000276:immunology; D018450:Disease Progression; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications* / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications*
chemical_list: D000974:Antibodies, Antinuclear; D001663:Bilirubin