LIVER DISEASE

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

C Rigamonti¹, L M Shand², M Feudjo³, C C Bunn², C M Black², C P Denton², A K Burroughs¹

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

Correspondence to:
Professor A K Burroughs
Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, Pond St, Hampstead, London NW3 2QG, UK; Andrew.burroughs{at}royalfree.nhs.uk

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 µmol/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 µmol/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.

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

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