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

(2018) HEPATOLOGY. 67(5). p.1920-1930
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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.
Keywords
PRIMARY SCLEROSING CHOLANGITIS, URSODEOXYCHOLIC ACID THERAPY, NORTH-EAST ENGLAND, BIOCHEMICAL RESPONSE, HISTOLOGICAL PROGRESSION, SYMPTOM PROGRESSION, CLINICAL-FEATURES, TREATED PATIENTS, FOLLOW-UP, CIRRHOSIS

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MLA
Murillo Perez, Fiorella, et al. “Milder Disease Stage in Patients with Primary Biliary Cholangitis over a 44-Year Period : A Changing Natural History.” HEPATOLOGY, vol. 67, no. 5, 2018, pp. 1920–30.
APA
Murillo Perez, F., Goet, J. C., Lammers, W. J., Gulamhusein, A., van Buuren, H. R., Ponsioen, C. Y., … Hansen, B. E. (2018). Milder disease stage in patients with primary biliary cholangitis over a 44-year period : a changing natural history. HEPATOLOGY, 67(5), 1920–1930.
Chicago author-date
Murillo Perez, Fiorella, Jorn C Goet, Willem J Lammers, Aliya Gulamhusein, Henk R van Buuren, Cyriel Y Ponsioen, Marco Carbone, et al. 2018. “Milder Disease Stage in Patients with Primary Biliary Cholangitis over a 44-Year Period : A Changing Natural History.” HEPATOLOGY 67 (5): 1920–30.
Chicago author-date (all authors)
Murillo Perez, Fiorella, Jorn C Goet, Willem J Lammers, Aliya Gulamhusein, Henk R van Buuren, Cyriel Y Ponsioen, Marco Carbone, Andrew Mason, Christophe Corpechot, Pietro Invernizzi, Marlyn J Mayo, Pier Maria Battezzati, Annarosa Floreani, Albert Pares, Frederik Nevens, Kris V Kowdley, Tony Bruns, George N Dalekos, Douglas Thorburn, Gideon Hirschfield, Nicholas F LaRusso, Keith D Lindor, Kalliopi Zachou, Raoul Poupon, Palak J Trivedi, Xavier Verhelst, Harry LA Janssen, and Bettina E Hansen. 2018. “Milder Disease Stage in Patients with Primary Biliary Cholangitis over a 44-Year Period : A Changing Natural History.” HEPATOLOGY 67 (5): 1920–1930.
Vancouver
1.
Murillo Perez F, Goet JC, Lammers WJ, Gulamhusein A, van Buuren HR, Ponsioen CY, et al. Milder disease stage in patients with primary biliary cholangitis over a 44-year period : a changing natural history. HEPATOLOGY. 2018;67(5):1920–30.
IEEE
[1]
F. Murillo Perez et al., “Milder disease stage in patients with primary biliary cholangitis over a 44-year period : a changing natural history,” HEPATOLOGY, vol. 67, no. 5, pp. 1920–1930, 2018.
@article{8541958,
  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.},
  author       = {Murillo Perez, Fiorella and Goet, Jorn C and Lammers, Willem J and Gulamhusein, Aliya and van Buuren, Henk R and Ponsioen, Cyriel Y and Carbone, Marco and Mason, Andrew and Corpechot, Christophe and Invernizzi, Pietro and Mayo, Marlyn J and Battezzati, Pier Maria and Floreani, Annarosa and Pares, Albert and Nevens, Frederik and Kowdley, Kris V and Bruns, Tony and Dalekos, George N and Thorburn, Douglas and Hirschfield, Gideon and LaRusso, Nicholas F and Lindor, Keith D and Zachou, Kalliopi and Poupon, Raoul and Trivedi, Palak J and Verhelst, Xavier and Janssen, Harry LA and Hansen, Bettina E},
  issn         = {0270-9139},
  journal      = {HEPATOLOGY},
  keywords     = {PRIMARY SCLEROSING CHOLANGITIS,URSODEOXYCHOLIC ACID THERAPY,NORTH-EAST ENGLAND,BIOCHEMICAL RESPONSE,HISTOLOGICAL PROGRESSION,SYMPTOM PROGRESSION,CLINICAL-FEATURES,TREATED PATIENTS,FOLLOW-UP,CIRRHOSIS},
  language     = {eng},
  number       = {5},
  pages        = {1920--1930},
  title        = {Milder disease stage in patients with primary biliary cholangitis over a 44-year period : a changing natural history},
  url          = {http://dx.doi.org/10.1002/hep.29717},
  volume       = {67},
  year         = {2018},
}

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