Professor Burroughs and colleagues from England evaluated the prognosis of primary biliary cirrhosis together with systemic sclerosis.
The research team used a primary biliary cirrhosis database of 580 patients, and identified 43 with primary biliary cirrhosis and systemic sclerosis.
At the initial visit, 2 patients with primary biliary cirrhosis alone were matched to each primary biliary cirrhosis-systemic sclerosis patient for serum bilirubin.
The researchers found that 93% of patients had limited cutaneous systemic sclerosis.
At diagnosis of primary biliary cirrhosis, the median age was 50 years.
The median levels of bilirubin were 17 µmol/l, and 41 g/l for albumin.
Liver diagnosis occurred a median 5 years after systemic sclerosis in 56% of patients.
In matched patients, median values at diagnosis were 53 years of age, bilirubin 12 µmol/l, and albumin 41 g/l.
|Risk of death from diagnosis was lower in primary biliary cirrhosis-systemic sclerosis patients|
The team reported that median follow up was 3 years for the primary biliary cirrhosis-systemic sclerosis patients.
Median follow-up for the patients with primary biliary cirrhosis alone was 5 years.
The risk of death from diagnosis, adjusting for sex, age, log bilirubin, and alkaline phosphatase was lower in primary biliary cirrhosis-systemic sclerosis.
The team observed that this was due to less transplantation.
The researchers noted that the rate of bilirubin increase was less in primary biliary cirrhosis-systemic sclerosis.
Overall survival was similar between the groups.
The team found that the mortality rate was 21% in patients with primary biliary cirrhosis-systemic sclerosis vs 11% in patients only with primary biliary cirrhosis.
Professor Burroughs' team concluded, “Liver disease has a slower progression in primary biliary cirrhosis-systemic sclerosis compared with matched patients with primary biliary cirrhosis alone.”