A 35-year follow-up of a large cohort of patients with primary biliary cirrhosis seen at a single centre



This article is corrected by:

  1. Errata: CORRIGENDA Volume 31, Issue 4, 588, Article first published online: 1 February 2011

Prof. Annarosa Floreani, Department of Surgical and Gastroenterological Sciences, University of Padova, Via Giustiniani 2, 35128 Padova, Italy
Tel: +39 049 8212894
Fax: +39 049 8760820
e-mail: annarosa.Floreani@unipd.it


Background: The natural history of primary biliary cirrhosis (PBC) is still debated.

Aims: 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.