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

• DOI: 10.1111/j.1478-3231.2010.02366.x
• Publication Date: 2010-11-09T03:14:29Z
• Authors: Annarosa Floreani; Diego Caroli; Angela Variola; Erik Rosa Rizzotto; Sara Antoniazzi; Maria Chiaramonte; Nora Cazzagon; Chiara Brombin; Luigi Salmaso; Vincenzo Baldo
• Keywords: Ursodeoxycholic acid; Primary Biliary Cirrhosis

Background: The natural history of primary biliary cirrhosis (PBC) is still debated. Aims: To evaluate: (i) long-term survival in a large cohort PBC patients observed prospectively at single centre and (ii) mortality relation to baseline characteristics ursodeoxycholic acid (UDCA) treatment. Methods: We considered all consecutive between 1973 2007 (327 subjects; 310 females, 17 males). Results: mean follow-up was 9.1±7.7 years. patients' age diagnosis for representative periods (1973–1980, 1981–1990, 1991–2000, 2001–2007) increased progressively from 47.7±1.5 53.2±1.2, 65.2±2.1 then 63.6±2.9 proportion asymptomatic 30 48% the last decade, while associated symptoms extrahepatic autoimmunity remained unchanged. Eighty (24.4%) died, 74 them because liver failure (12 developed hepatocellular carcinoma); nine underwent transplantation. From 1988 onwards, were treated with UDCA (n=288). death sample as whole 67.2±1.3 probability 20 years 82% histological stages I–II entry, 64% those stage III 42% IV (P=0.0007). Mortality significantly reduced (P=0.012), whereas it independently oesophageal varices (P=0.015). Patients had better prognosis than untreated, irrespective stage. Early subjects good response have an 85% chance Conclusions: clinical presentation has been changing over Its early detection treatment improve related rates.