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A 35‐year follow‐up of a large cohort of patients with primary biliary cirrhosis seen at a single centre
Author(s) -
Floreani Annarosa,
Caroli Diego,
Variola Angela,
Rizzotto Erik Rosa,
Antoniazzi Sara,
Chiaramonte Maria,
Cazzagon Nora,
Brombin Chiara,
Salmaso Luigi,
Baldo Vincenzo
Publication year - 2011
Publication title -
liver international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.873
H-Index - 110
eISSN - 1478-3231
pISSN - 1478-3223
DOI - 10.1111/j.1478-3231.2010.02366.x
Subject(s) - medicine , ursodeoxycholic acid , gastroenterology , asymptomatic , primary biliary cirrhosis , cohort , liver transplantation , cirrhosis , natural history , hepatocellular carcinoma , stage (stratigraphy) , varices , transplantation , surgery , paleontology , biology
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.