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Gut. 2006 Mar;55(3):388-94. Epub 2005 Sep 8.

Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis.

Author information

  • 1Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, Pond St, Hampstead, London NW3 2QG, UK.

Abstract

BACKGROUNDS AND AIMS:

To evaluate the prognosis of primary biliary cirrhosis (PBC) together with systemic sclerosis (SSc), as this is unknown.

METHODS AND 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.

PMID:
16150855
[PubMed - indexed for MEDLINE]
PMCID:
PMC1856066
Free PMC Article

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