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

Cristina Rigamonti ¹, Lynne M Shand ², Maurille Feudjo ³, Chris C Bunn ², Carol M Black ², Christopher P Denton ² and Andrew K Burroughs ^1*

¹ Royal Free Hospital, Liver Transplantation and Hepatobiliary Medicine, United Kingdom
² Royal Free Hospital, Centre for Rheumatology, United Kingdom
³ London School of Hygiene and Tropical Medicine, Medical Statistics Unit, United Kingdom

^* To whom correspondence should be addressed. E-mail: andrew.burroughs{at}royalfree.nhs.uk.

Accepted 31 August 2005

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: 2 patients with PBC alone were matched to each PBC-SSc patient for serum bilirubin concentration at the initial visit. Forty (93%) had limited cutaneous SSc. At diagnosis of PBC median values were: 49.7 years, bilirubin 17 µ mol/L, albumin 40.5 g/L. The liver diagnosis occurred a median 4.9 years after SSc in 24 (56%). In matched patients, median values at diagnosis were: 53.2 years, bilirubin 12 µmol/L, albumin 41 g/L. Median follow-up was similar: 3.16 years (PBC-SSc), 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); deaths: 9 (21%) in PBC-SSc (6 SSc-related and 2 liver-related), 9 (11%) in PBC alone (6 liver-related).

Conclusions: Liver disease has a slower progression in PBC-SSc, compared to matched patients with PBC alone.

Keywords: anticentromere antibody, antimitochondrial antibody, autoimmune disease, liver transplantation, survival

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