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

Rigamonti, C; Shand, LM; Feudjo, M; Bunn, CC; Black, CM; Denton, CP; Burroughs, AK; (2006) Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis. Gut , 55 (3) pp. 388-394. 10.1136/gut.2005.075002.

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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.

Type: Article
Title: Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis.
Location: England
DOI: 10.1136/gut.2005.075002
Keywords: Adult, Aged, Aged, 80 and over, Antibodies, Antinuclear, Bilirubin, Centromere, Disease Progression, Epidemiologic Methods, Female, Humans, Liver Cirrhosis, Biliary, Liver Transplantation, Male, Middle Aged, Prognosis, Scleroderma, Systemic
UCL classification: UCL > School of Life and Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Medical Sciences > Medicine (Division of)
URI: http://discovery.ucl.ac.uk/id/eprint/493681
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