PT  - JOURNAL ARTICLE
AU  - C Rigamonti
AU  - L M Shand
AU  - M Feudjo
AU  - C C Bunn
AU  - C M Black
AU  - C P Denton
AU  - A K Burroughs
TI  - Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis
AID  - 10.1136/gut.2005.075002
DP  - 2006 Mar 01
TA  - Gut
PG  - 388--394
VI  - 55
IP  - 3
4099  - http://gut.bmj.com/content/55/3/388.short
4100  - http://gut.bmj.com/content/55/3/388.full
SO  - Gut2006 Mar 01; 55
AB  - 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.