RESUMO
BACKGROUND: Primary biliary cirrhosis is a rare cholestasis liver disease affecting the women of mature age,it association with Autoimmune hepatitis defined the overlap syndrome. AIM: It was to determine epidemiological, clinic-biological, immunological, histological, therapeutic and evolutive characteristics of PBC and to compare them to those in subjects having an overlap syndrome. METHODS: It is a retrospective study grouping all the cases of PBC hospitalized over a period of 15 years (1995-2009) in the hepatogastro-enterology department at Sahloul hospital in Sousse. RESULTS: Thirty six cases were grouped over the period of the study. They were all women. The mean age was 54.5 years (28-79). The disease was symptomatic at the moment of diagnosis in 75% of the cases. The functional signs revealing the disease were principally asthenia, present in 69.4% of the cases, followed by jaundice and prurit. Cholestasis was constant in all the patients, stage III of scheuer was the most frequent in the patients (25%) followed by stage IV (22%). OS was present in 1/3 of the patients; the treatment is based on the prescription of urso-desoxycholic Acid associated with corticoids and with immunosuppressors in the case of OS. The average survival of the patient having PBC and OS was comparable. It was respectively of 61.8 and 55.9 months. CONCLUSION: Our results as well as the literature reveal the rarety of this disease and its predominance in women. PBC has to be diagnosed at an early stage to guarantee a better response and a better survival of the patients.
