title: [Primary biliary cirrhosis: a thirteen years experience].
abstract: BACKGROUND
  Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure.
  
  
  OBJECTIVE
  To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC.
  
  
  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.
  
  
  RESULTS
  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. Sjögren 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.
authors.affiliation: Sección de Gastroenterología, Hospital Clínico, Universidad de Chile, Santiago, Chile.
authors.forename: José Miguel
authors.initials: JM
authors.lastname: Valera M
authors.forename: Gladys
authors.initials: G
authors.lastname: Smok S
authors.forename: Jaime
authors.initials: J
authors.lastname: Poniachik T
authors.forename: Danny
authors.initials: D
authors.lastname: Oksenberg R
authors.forename: Guillermo
authors.initials: G
authors.lastname: Silva P
authors.forename: Mario
authors.initials: M
authors.lastname: Ferrario B
authors.forename: Erwin
authors.initials: E
authors.lastname: Buckel G
authors.forename: Javier
authors.initials: J
authors.lastname: Brahm B
mesh_terms: D000328:Adult; D000368:Aged; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007037:Hypothyroidism / Q000150:complications; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid