[Primary biliary cirrhosis: clinical features and survival of a Mexican population]

PBC progresses to cirrhosis and results in death due to liver failure or bleeding portal hypertension. Data of the clinical characteristics and survival of PBC patients allows the assessment of therapeutical alternatives as well as the establishment of inclusion criteria for liver transplantation.

One hundred and twenty patients with histological diagnosis of PBC, admitted from 1972 to 1992, were selected with the purpose of studying the clinical and biochemical characteristics and survival.

Patients who underwent liver transplant or those who had an incomplete follow-up were excluded.

Therefore only 80 patients were included: these were seventy five women and five men, with mean age 46 +/- 11 years (X +/- SD) to whom demographic data, biochemical analysis, liver function (Child-Pugh) and liver damage (Ludwig) were recorded at the time of histological diagnosis, which was considered zero for calculating the survival (Kaplan Meier). The most common symptoms at diagnosis were pruritus in 63 patients, jaundice in 48, asthenia and adynamia in 55 patients. Eight cases were asymptomatic. According to Child-Pugh's classification, patients were grouped as follows: forty in stage A, 29 in B, and three in C; and according to liver damage (Ludwig), 8 in grade I, 28 in grade II, 22 in grade III and 14 in grade IV. The most frequent clinical associations were Sjögren's syndrome, in 30% of patients, although one case was associated to progressive muscular dystrophy and another one to multiple myeloma and hypothyroidism; in 58.7% of the cases, antimitochondrial antibodies were negative. One year survival was 75%, five years 44%, and seven years 13%.

The most important characteristics of the studied patients were elevated percentage of negative antimitochondrial antibodies and short survival. it is important to impel the development of liver transplantation as the only mean to improve survival.