A 35-year follow-up of a large cohort of patients with primary biliary cirrhosis seen at a single centre.
BACKGROUND
The natural history of primary biliary cirrhosis (PBC) is still debated.


OBJECTIVE
To evaluate: (i) long-term survival in a large cohort of PBC patients observed prospectively at a single centre and (ii) mortality in relation to baseline characteristics and ursodeoxycholic acid (UDCA) treatment.


METHODS
We considered all consecutive patients between 1973 and 2007 (327 subjects; 310 females, 17 males).


RESULTS
The mean follow-up was 9.1±7.7 years. The patients' age at diagnosis for representative periods (1973-1980, 1981-1990, 1991-2000, 2001-2007) increased progressively from 47.7±1.5 to 53.2±1.2, to 65.2±2.1 and then 63.6±2.9 years. The proportion of asymptomatic patients at diagnosis increased from 30 to 48% in the last decade, while associated symptoms of extrahepatic autoimmunity remained unchanged. Eighty patients (24.4%) died, 74 of them because of liver failure (12 patients developed hepatocellular carcinoma); nine patients underwent liver transplantation. From 1988 onwards, all patients were treated with UDCA (n=288). The mean age at death for the sample as a whole was 67.2±1.3 years. The survival probability at 20 years was 82% for patients with histological stages I-II at entry, 64% for those with stage III and 42% for those with stage IV (P=0.0007). Mortality was significantly reduced in patients treated with UDCA (P=0.012), whereas it was independently associated with oesophageal varices (P=0.015). Patients treated with UDCA had a better prognosis than those untreated, irrespective of the histological stage. Early treated subjects with a good response to UDCA have an 85% chance of survival at 20 years.


CONCLUSIONS
The clinical presentation of PBC has been changing over the years. Its early detection and early treatment improve the related survival rates.
Department of Surgical and Gastroenterological Sciences, University of Padova, Padova, Italy. annarosa.Floreani@unipd.it
Annarosa
A
Floreani
Diego
D
Caroli
Angela
A
Variola
Erik Rosa
ER
Rizzotto
Sara
S
Antoniazzi
Maria
M
Chiaramonte
Nora
N
Cazzagon
Chiara
C
Brombin
Luigi
L
Salmaso
Vincenzo
V
Baldo
D002423:Cause of Death; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D017093:Liver Failure / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid