Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history

on behalf of the GLOBAL PBC Study Group

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Changes over time in the presenting features and clinical course of patients with primary biliary cholangitis are poorly described. We sought to describe temporal trends in patient and disease characteristics over a 44-year period across a large international primary biliary cholangitis cohort of 4,805 patients diagnosed between 1970 and 2014, from 17 centers across Europe and North America. Patients were divided into five cohorts according to their year of diagnosis: 1970-1979 (n = 143), 1980-1989 (n = 858), 1990-1999 (n = 1,754), 2000-2009 (n = 1,815), and ≥2010 (n = 235). Age at diagnosis, disease stage, response to ursodeoxycholic acid, and clinical outcomes were compared. Mean age at diagnosis increased incrementally by 2-3 years per decade from 46.9 ± 10.1 years in the 1970s to 57.0 ± 12.1 years from 2010 onward (P < 0.001). The female to male ratio (9:1) and antimitochondrial antibody positivity (90%) were not significantly variable. The proportion of patients presenting with mild biochemical disease (according to Rotterdam staging) increased from 41.3% in the 1970s to 72.2% in the 1990s (P < 0.001) and remained relatively stable thereafter. Patients with a mild histological stage at diagnosis increased from 60.4% (1970-1989) to 76.5% (1990-2014) (P < 0.001). Correspondingly, response to ursodeoxycholic acid according to Paris-I criteria increased; 51.7% in the 1970s and 70.5% in the 1990s (P < 0.001). Recent decades were also characterized by lower decompensation rates (18.5% in the 1970s to 5.8% in the 2000s, P < 0.001) and higher 10-year transplant-free survival (48.4%, 68.7%, 79.7%, and 80.1% for each respective cohort; P < 0.001). Conclusion: In recent decades, a pattern of primary biliary cholangitis presentation consistent with an older age at diagnosis alongside reduced disease severity has been noted; the observed trends may be explained by an increase in routine testing of liver function and/or a changing environmental trigger. (Hepatology 2018;67:1920-1930).

Original languageEnglish (US)
Pages (from-to)1920-1930
Number of pages11
JournalHepatology
Volume67
Issue number5
DOIs
StatePublished - May 1 2018

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Cholangitis
Natural History
Ursodeoxycholic Acid
Paris
Gastroenterology
North America
Transplants
Survival
Antibodies
Liver

ASJC Scopus subject areas

  • Hepatology

Cite this

Milder disease stage in patients with primary biliary cholangitis over a 44-year period : A changing natural history. / on behalf of the GLOBAL PBC Study Group.

In: Hepatology, Vol. 67, No. 5, 01.05.2018, p. 1920-1930.

Research output: Contribution to journalArticle

on behalf of the GLOBAL PBC Study Group. / Milder disease stage in patients with primary biliary cholangitis over a 44-year period : A changing natural history. In: Hepatology. 2018 ; Vol. 67, No. 5. pp. 1920-1930.
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title = "Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history",
abstract = "Changes over time in the presenting features and clinical course of patients with primary biliary cholangitis are poorly described. We sought to describe temporal trends in patient and disease characteristics over a 44-year period across a large international primary biliary cholangitis cohort of 4,805 patients diagnosed between 1970 and 2014, from 17 centers across Europe and North America. Patients were divided into five cohorts according to their year of diagnosis: 1970-1979 (n = 143), 1980-1989 (n = 858), 1990-1999 (n = 1,754), 2000-2009 (n = 1,815), and ≥2010 (n = 235). Age at diagnosis, disease stage, response to ursodeoxycholic acid, and clinical outcomes were compared. Mean age at diagnosis increased incrementally by 2-3 years per decade from 46.9 ± 10.1 years in the 1970s to 57.0 ± 12.1 years from 2010 onward (P < 0.001). The female to male ratio (9:1) and antimitochondrial antibody positivity (90{\%}) were not significantly variable. The proportion of patients presenting with mild biochemical disease (according to Rotterdam staging) increased from 41.3{\%} in the 1970s to 72.2{\%} in the 1990s (P < 0.001) and remained relatively stable thereafter. Patients with a mild histological stage at diagnosis increased from 60.4{\%} (1970-1989) to 76.5{\%} (1990-2014) (P < 0.001). Correspondingly, response to ursodeoxycholic acid according to Paris-I criteria increased; 51.7{\%} in the 1970s and 70.5{\%} in the 1990s (P < 0.001). Recent decades were also characterized by lower decompensation rates (18.5{\%} in the 1970s to 5.8{\%} in the 2000s, P < 0.001) and higher 10-year transplant-free survival (48.4{\%}, 68.7{\%}, 79.7{\%}, and 80.1{\%} for each respective cohort; P < 0.001). Conclusion: In recent decades, a pattern of primary biliary cholangitis presentation consistent with an older age at diagnosis alongside reduced disease severity has been noted; the observed trends may be explained by an increase in routine testing of liver function and/or a changing environmental trigger. (Hepatology 2018;67:1920-1930).",
author = "{on behalf of the GLOBAL PBC Study Group} and {Murillo Perez}, {Carla F.} and Goet, {Jorn C.} and Lammers, {Willem J.} and Aliya Gulamhusein and {van Buuren}, {Henk R.} and Ponsioen, {Cyriel Y.} and Marco Carbone and Andrew Mason and Christophe Corpechot and Pietro Invernizzi and Mayo, {Marlyn J.} and Battezzati, {Pier Maria} and Annarosa Floreani and Albert Pares and Frederik Nevens and Kowdley, {Kris V.} and Tony Bruns and Dalekos, {George N.} and Douglas Thorburn and Gideon Hirschfield and {La Russo}, {Nicholas F} and Lindor, {Keith D.} and Kalliopi Zachou and Raoul Poupon and Trivedi, {Palak J.} and Xavier Verhelst and Janssen, {Harry L.A.} and Hansen, {Bettina E.}",
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T1 - Milder disease stage in patients with primary biliary cholangitis over a 44-year period

T2 - A changing natural history

AU - on behalf of the GLOBAL PBC Study Group

AU - Murillo Perez, Carla F.

AU - Goet, Jorn C.

AU - Lammers, Willem J.

AU - Gulamhusein, Aliya

AU - van Buuren, Henk R.

AU - Ponsioen, Cyriel Y.

AU - Carbone, Marco

AU - Mason, Andrew

AU - Corpechot, Christophe

AU - Invernizzi, Pietro

AU - Mayo, Marlyn J.

AU - Battezzati, Pier Maria

AU - Floreani, Annarosa

AU - Pares, Albert

AU - Nevens, Frederik

AU - Kowdley, Kris V.

AU - Bruns, Tony

AU - Dalekos, George N.

AU - Thorburn, Douglas

AU - Hirschfield, Gideon

AU - La Russo, Nicholas F

AU - Lindor, Keith D.

AU - Zachou, Kalliopi

AU - Poupon, Raoul

AU - Trivedi, Palak J.

AU - Verhelst, Xavier

AU - Janssen, Harry L.A.

AU - Hansen, Bettina E.

PY - 2018/5/1

Y1 - 2018/5/1

N2 - Changes over time in the presenting features and clinical course of patients with primary biliary cholangitis are poorly described. We sought to describe temporal trends in patient and disease characteristics over a 44-year period across a large international primary biliary cholangitis cohort of 4,805 patients diagnosed between 1970 and 2014, from 17 centers across Europe and North America. Patients were divided into five cohorts according to their year of diagnosis: 1970-1979 (n = 143), 1980-1989 (n = 858), 1990-1999 (n = 1,754), 2000-2009 (n = 1,815), and ≥2010 (n = 235). Age at diagnosis, disease stage, response to ursodeoxycholic acid, and clinical outcomes were compared. Mean age at diagnosis increased incrementally by 2-3 years per decade from 46.9 ± 10.1 years in the 1970s to 57.0 ± 12.1 years from 2010 onward (P < 0.001). The female to male ratio (9:1) and antimitochondrial antibody positivity (90%) were not significantly variable. The proportion of patients presenting with mild biochemical disease (according to Rotterdam staging) increased from 41.3% in the 1970s to 72.2% in the 1990s (P < 0.001) and remained relatively stable thereafter. Patients with a mild histological stage at diagnosis increased from 60.4% (1970-1989) to 76.5% (1990-2014) (P < 0.001). Correspondingly, response to ursodeoxycholic acid according to Paris-I criteria increased; 51.7% in the 1970s and 70.5% in the 1990s (P < 0.001). Recent decades were also characterized by lower decompensation rates (18.5% in the 1970s to 5.8% in the 2000s, P < 0.001) and higher 10-year transplant-free survival (48.4%, 68.7%, 79.7%, and 80.1% for each respective cohort; P < 0.001). Conclusion: In recent decades, a pattern of primary biliary cholangitis presentation consistent with an older age at diagnosis alongside reduced disease severity has been noted; the observed trends may be explained by an increase in routine testing of liver function and/or a changing environmental trigger. (Hepatology 2018;67:1920-1930).

AB - Changes over time in the presenting features and clinical course of patients with primary biliary cholangitis are poorly described. We sought to describe temporal trends in patient and disease characteristics over a 44-year period across a large international primary biliary cholangitis cohort of 4,805 patients diagnosed between 1970 and 2014, from 17 centers across Europe and North America. Patients were divided into five cohorts according to their year of diagnosis: 1970-1979 (n = 143), 1980-1989 (n = 858), 1990-1999 (n = 1,754), 2000-2009 (n = 1,815), and ≥2010 (n = 235). Age at diagnosis, disease stage, response to ursodeoxycholic acid, and clinical outcomes were compared. Mean age at diagnosis increased incrementally by 2-3 years per decade from 46.9 ± 10.1 years in the 1970s to 57.0 ± 12.1 years from 2010 onward (P < 0.001). The female to male ratio (9:1) and antimitochondrial antibody positivity (90%) were not significantly variable. The proportion of patients presenting with mild biochemical disease (according to Rotterdam staging) increased from 41.3% in the 1970s to 72.2% in the 1990s (P < 0.001) and remained relatively stable thereafter. Patients with a mild histological stage at diagnosis increased from 60.4% (1970-1989) to 76.5% (1990-2014) (P < 0.001). Correspondingly, response to ursodeoxycholic acid according to Paris-I criteria increased; 51.7% in the 1970s and 70.5% in the 1990s (P < 0.001). Recent decades were also characterized by lower decompensation rates (18.5% in the 1970s to 5.8% in the 2000s, P < 0.001) and higher 10-year transplant-free survival (48.4%, 68.7%, 79.7%, and 80.1% for each respective cohort; P < 0.001). Conclusion: In recent decades, a pattern of primary biliary cholangitis presentation consistent with an older age at diagnosis alongside reduced disease severity has been noted; the observed trends may be explained by an increase in routine testing of liver function and/or a changing environmental trigger. (Hepatology 2018;67:1920-1930).

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