| Abstract | dc.description.abstract | Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic
disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological
features, treatment and outcome of a group of patients with PBC. Material and methods:
Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with
PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver
transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at
presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in
56%. No clinical or laboratory differences were observed between symptomatic patients or those with
positive antimitochondrial antibodies and the rest of the study group. Sjogren syndrome was present
in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61%
had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen
patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has
been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred.
Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the
incidence of complications and the use of costly treatments. | en |
