Clinical features at diagnosis of primary biliary cirrhosis (PBC) in 43 patients with PBC-systemic sclerosis (PBC-SSc) and 82 with PBC alone matched by serum bilirubin at referral to the Royal Free Hospital, and in 13 patients with PBC who were anticentromere antibody positive but had no features of SSc†
| PBC-SSc (n = 43) | PBC (n = 82) | PBC-ACA positive (n = 13) | |
|---|---|---|---|
| Values are number (%). | |||
| †Comparison was done between patients with PBC-SSc and patients with PBC alone (that is, first two columns). | |||
| *p = 0.03 (Pearson χ2 test), 95% 95% confidence interval (CI) 0.34–0.98. | |||
| ‡p = 0.047 (Pearson χ2 test), 95% CI 0.26–1.06. | |||
| §p = 0.03 (Pearson χ2 test), 95% CI 1.29–3.79. | |||
| Asymptomatic (no liver symptoms) | 17 (39%) | 22 (27%) | 3 (23%) |
| Symptoms at diagnosis | |||
| Pruritus | 14 (33%)* | 43 (52%)* | 4 (31%) |
| Jaundice | 4 (9%) | 16 (19.%) | 2 (15%) |
| Fatigue | 7 (16%)‡ | 27 (33%)‡ | 2 (15%) |
| Abdominal pain | 6 (14%) | 13 (16%) | 4 (31%) |
| Diarrhoea | 5 (12%)§ | 2 (2%)§ | 1 (8%) |
| Oesophageal varices (at endoscopy) | 2 (5%) | 4 (5%) | 1 (8%) |
| Bleeding varices | 2 (5%) | 2 (2%) | 1 (8%) |
| Haematemesis or maelena | 2 (5%) | 3 (4%) | 1 (8%) |
| Bone pain | 6 (14%) | 15 (18%) | 1 (8%) |
| Fever | 0 (0%) | 2 (2%) | 0 (0%) |
| Weight loss | 2 (5%) | 10 (12%) | 0 (0%) |
| Amenorrhoea | 4 (10%) | 6 (7%) | 2 (15%) |
| Dry eyes and/or dry mouth | 8 (20%) | 9 (11%) | 3 (23%) |
| Ankle swelling | 1 (2%) | 1 (1%) | 0 (0%) |
| Abdominal swelling | 0 (0%) | 1 (1%) | 0 (0%) |
| Signs at diagnosis | |||
| Jaundice | 5 (12%) | 17 (21%) | 1 (8%) |
| Hepatomegaly | 10 (27%) | 27 (33%) | 5 (38%) |
| Splenomegaly | 7 (20%) | 11 (13%) | 3 (23%) |
| Hyperpigmentation | 2 (5%) | 9 (11%) | 2 (15%) |
| Xanthomata/xantelasma | 0 (0%) | 4 (5%) | 0 (0%) |
| Spider naevi | 2 (5%) | 6 (7%) | 1 (8%) |
| Ankle swelling | 1 (2%) | 1 (1%) | 0 (0%) |
| Ascites | 0 (0%) | 1 (1%) | 0 (0%) |
| Portosystemic encephalopathy | 0 (0%) | 0 (0%) | 0 (0%) |
| Muscular wasting | 0 (0%) | 2 (2%) | 0 (0%) |
| Finger clubbing | 0 (0%) | 1 (1%) | 0 (0%) |
| Pyrexia | 0 (0%) | 2 (2%) | 0 (0%) |