Table 1

 Primary biliary cirrhosis-systemic sclerosis (PBC-SSc) patients matched with 82 with PBC alone by serum bilirubin, at referral to the Royal Free Hospital

PBC-SScPBC
Values are number (%) or median (range).
AMA, antimitochondrial antibody; ACA, anticentromere antibody; UDCA, ursodeoxycholic acid
*Mann-Whitney U test was done for comparison of the two cohorts. No significant differences were observed.
†Two sample t test with equal variances was done using log transformed values for comparison of the two cohorts. No significant differences were observed.
No of patients4382
    Females40 (93%)76 (93%)
Age at diagnosis of PBC (y)49.7 (22.1–70.1)*53.2 (25.9–82.1)*
AMA positive40 (93%)77 (94%)
ACA positive30 (70%)0 (0%)
Bilirubin at diagnosis (μmol/l)17 (4–109)†12 (2–120)†
Albumin at diagnosis (g/l)40.5 (29–49)†41 (31–48)†
Alkaline phosphatase at diagnosis (U/l)334 (74–1038)†403 (89–2300)†
UDCA treatment22 (51%)43 (52%)
UDCA treatment at dosage ⩾13 mg/kg/day12 (28%)35 (43%)
Start of UDCA (y after diagnosis of PBC)2.1 (0–10.2)0.8 (0–10.3)