Excellent outcome in patients with primary biliary cholangitis in Northwest Italy followed up for up to 30 years.

Autor:	Rigamonti C; Department of Translational Medicine, Università del Piemonte Orientale, Division of Internal Medicine, AOU Maggiore della Carità, Novara., De Benedittis C; Department of Translational Medicine, Università del Piemonte Orientale, Division of Internal Medicine, AOU Maggiore della Carità, Novara., Labanca S; Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS Ospedale Policlinico San Martino, Genoa., Vanni E; SC Gastroenterology, AOU Città della Salute e della Scienza, Turin., Morgando A; SC Gastroenterology, AOU Città della Salute e della Scienza, Turin., Manfredi GF; Department of Translational Medicine, Università del Piemonte Orientale, Division of Internal Medicine, AOU Maggiore della Carità, Novara., Azzolina D; Department of Environment and Prevention Sciences, University of Ferrara, Ferrara, Italy., Cittone MG; Department of Translational Medicine, Università del Piemonte Orientale, Division of Internal Medicine, AOU Maggiore della Carità, Novara., Giannini EG; Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS Ospedale Policlinico San Martino, Genoa., Saracco GM; SC Gastroenterology, AOU Città della Salute e della Scienza, Turin., Pirisi M; Department of Translational Medicine, Università del Piemonte Orientale, Division of Internal Medicine, AOU Maggiore della Carità, Novara.
Jazyk:	angličtina
Zdroj:	European journal of gastroenterology & hepatology [Eur J Gastroenterol Hepatol] 2023 Aug 01; Vol. 35 (8), pp. 899-906. Date of Electronic Publication: 2023 Jun 06.
DOI:	10.1097/MEG.0000000000002582
Abstrakt:	Objective: Primary biliary cholangitis (PBC) is a rare chronic autoimmune cholangiopathy, characterized by a variable course and response to treatment. We aimed to describe long-term outcomes of PBC patients referred to three academic centres in Northwest Italy. Methods: This is an ambispective cohort study of PBC patients (retrospective component: diagnosis before 1 January 2019; prospective component: thereafter), including 302 patients: 101 (33%) followed up in Novara, 86 (28%) in Turin, 115 (38%) in Genoa. Clinical features at diagnosis, biochemical response to therapy and survival were analyzed. Results: Among the 302 patients (88% women, median age 55 years, median follow-up 75 months), alkaline phosphatase (ALP) levels significantly decreased during treatment with ursodeoxycholic acid (UDCA, P < 0.0001) and obeticholic acid (P < 0.0001). At multivariate analysis, ALP at diagnosis was predictive of 1-year biochemical response to UDCA [odds ratio 3.57, 95% confidence interval (CI) 1.4-9, P < 0.001]. Estimated median survival free of liver transplantation and hepatic complications was 30 years (95% CI 19-41). Bilirubin level at diagnosis was the only independent risk factor for the combined outcome of death, transplantation or hepatic decompensation (hazard ratio, 1.65, 95% CI 1.66-2.56, P = 0.02). Patients presenting with total bilirubin at diagnosis ≥0.6 times the upper normal limit (ULN) had a significantly lower 10-year survival compared to those with bilirubin <0.6 times ULN (63% vs. 97%, P < 0.0001). Conclusion: In PBC, both short-term response to UDCA and long-term survival can be predicted by simple conventional biomarkers of disease severity, obtained at diagnosis. (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)
Databáze:	MEDLINE
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