title: [Primary biliary cirrhosis: clinical and epidemiological features in an Uruguayan population].
abstract: BACKGROUND
  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.
  
  
  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.
authors: Clínica de Gastroenterología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay. adriana@sanchez.com.uy
authors: Adriana
authors: A
authors: Sánchez
authors: Clínica de Gastroenterología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay.
authors: Nelia
authors: N
authors: Hernández
authors: Clínica de Gastroenterología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay.
authors: Daniela
authors: D
authors: Chiodi
authors: Clínica de Gastroenterología, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay.
authors: Joaquín
authors: J
authors: Berrueta
authors: Servicio de Gastroenterología, Hospital Maciel, Montevideo, Uruguay.
authors: Gabriela
authors: G
authors: Robaina
authors: Servicio de Gastroenterología, Hospital Maciel, Montevideo, Uruguay.
authors: Carmen
authors: C
authors: Pollio
authors: Unidad Hepatobiliopancreática, Asociación Española, Montevideo, Uruguay.
authors: Germán
authors: G
authors: Mescia
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D001706:Biopsy; D015331:Cohort Studies; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000175:diagnosis / Q000401:mortality; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014583:Uruguay / Q000453:epidemiology
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies