[Primary biliary cirrhosis: a thirteen years experience].
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.
Valera M
José Miguel
JM
Sección de Gastroenterología, Hospital Clínico, Universidad de Chile, Santiago, Chile.
Smok S
Gladys
G
Poniachik T
Jaime
J
Oksenberg R
Danny
D
Silva P
Guillermo
G
Ferrario B
Mario
M
Buckel G
Erwin
E
Brahm B
Javier
J
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
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid