Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis
- 1Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, Hampstead, London, UK
- 2Centre for Rheumatology, Royal Free Hospital, Hampstead, London, UK
- 3London 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;
- Accepted 31 August 2005
- Revised 31 July 2005
- Published Online First 8 September 2005
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.
- ACA, anticentromere antibody
- AMA, antimitochondrial antibody
- ANA, antinuclear antibody
- CREST, calcinosis, Raynaud’s phenomenon, oesophageal dysfunction, sclerodactyly, telangiectasia
- dcSSc, diffuse cutaneous systemic sclerosis
- HCC, hepatocellular carcinoma
- lcSSc, limited cutaneous systemic sclerosis
- nRNP, nuclear ribonucleoprotein
- PBC, primary biliary cirrhosis
- PSE, portosystemic encephalopathy
- SBP, spontaneous bacterial peritonitis
- Scl70, antitopoisomerase I
- SSc, systemic sclerosis
- UDCA, ursodeoxycholic acid
- liver transplantation
- antimitochondrial antibody
- anticentromere antibody
- autoimmune disease
Published online first 8 September 2005
Conflict of interest: None declared.