RESUMEN
INTRODUCTION: Primary biliary cirrhosis (PBC) is a chronic cholestatic, autoimmune, liver disease produced by inflammation and destruction of the interlobular bile ducts. It is more frequent among female patients and is usually diagnosed in the fifth decade of life. OBJECTIVE: Our objective was to describe the clinical and epidemiological characteristics of patients with PBC in Uruguay. MATERIAL AND METHODS: This descriptive study included patients from 3 medical centers diagnosed with PBC in the period January 2002 to September 2011. The diagnosis was based on the presence of at least two of the following requirements: cholestasis, antimitochondrial antibodies (AMA) (or AMA subtype 2) or positive antinuclear antibodies (ANA) (anticentromere pattern) and compatible biopsy. Data recorded were sex, age, symptoms, related illness, laboratory results, images and histology at the moment of the diagnosis. RESULTS: We included 81 patients, 94
were women and the mean age was 56 years old (range: 31 to 79 years old). Symptoms were present in 59 patients (73
) and pruritus, found in 51 of them (86
), was the most frequent symptom. Positive AMA was found in 84
of cases. Histological study was available in 35 patients (43
) and 13 of them (37
) had cirrhosis. The mean survival according to the presence or absence of cirrhosis was 9.17 years (95
confidence interval: 6.79-11.56) and 10.7 years (95
confidence interval: 9.27-12.14), respectively (P = 0.03). CONCLUSIONS: Female predominance and frequent association with other autoimmune diseases were confirmed in this group. Although there was a high percentage of symptomatic and cirrhotic patients at diagnosis, only the presence of cirrhosis was associated with a lower survival.