Increasing Prevalence of Primary Biliary Cholangitis and Reduced Mortality With Treatment

Fibrotic Liver Disease Consortium Investigators

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Background & Aims: There are few data from longitudinal studies of trends in primary biliary cholangitis (PBC) among patients under routine clinical care in the United States. We collected data from the Fibrotic Liver Disease consortium to investigate changes in the incidence and prevalence of PBC and the effects of patient demographics, clinical features, and treatment on mortality. Methods: We collected demographic and clinical data for the general patient population as well as PBC patients receiving care from 11 health systems in different regions of the United States (Northeast, Midwest, Northwest, and South) from January 1, 2003, through December 31, 2014. Annual percentage changes in PBC prevalence and incidence were estimated using join-point Poisson regression. Differences based on race, age, and gender were calculated with rate ratios. All-cause mortality was estimated using Cox regression with adjustment for patient characteristics and treatment with ursodeoxycholic acid (UDCA). Propensity scores were used to adjust for treatment selection bias. Analyses were adjusted by geographic regions. Results: In our racially diverse cohort of 3488 patients with PBC (21% Hispanic, 8% African American, 7% Asian American), 70% had ever received UDCA. From 2006 through 2014, the prevalence of PBC increased from 21.7 to 39.2 per 100,000 persons. Adjusted annual percentage changes in prevalence differed among age groups (≤40 y, 41–50 y, 51–60 y, 61–70 y, and >70 y), ranging from 3.0% to 7.5% (P <.05). Incidence did not change significantly during the study period (4.2 vs 4.3 per 100,000 person-years in 2006 and 2014, respectively; P =.98). Ratios of prevalence for women vs men (3.9:1) and incidence for women vs men (3.2:1) were consistent over the study period. Among African Americans, the prevalence of PBC increased from 16.9 to 30.8 per 100,000 during the study period, and annual incidence ranged from 2.6 to 6.6 per 100,000 person-years. In adjusted analyses, an increased level of alkaline phosphatase at baseline was associated with significantly higher mortality (adjusted hazard ratios [aHR], 1.24; 95% CI, 1.04–1.48 for patients with levels 1–2 times the upper limit of normal and aHR, 2.27; 95% CI, 1.88–2.73 for patients with levels more than 3 times the upper limit of normal). UDCA treatment was associated with significantly reduced mortality (aHR, 0.57; 95% CI, 0.52–0.64). Conclusions: In an analysis of data from patients receiving routine clinical care in Fibrotic Liver Disease Consortium health systems, we found that the prevalence of PBC increased from 2004 through 2014, despite steady incidence. Patient demographic and clinical characteristics, as well as UDCA treatment, affected mortality.

Original languageEnglish (US)
Pages (from-to)1342-1350.e1
JournalClinical Gastroenterology and Hepatology
Volume16
Issue number8
DOIs
StatePublished - Aug 1 2018

Fingerprint

Cholangitis
Mortality
Ursodeoxycholic Acid
Incidence
Therapeutics
Demography
African Americans
Liver Diseases
Propensity Score
Asian Americans
Selection Bias
Health
Hispanic Americans
Alkaline Phosphatase
Longitudinal Studies
Patient Care
Age Groups

Keywords

  • Autoimmune Disease
  • Epidemiology
  • FOLD Consortium
  • Gender
  • Racial Disparities

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

Cite this

Increasing Prevalence of Primary Biliary Cholangitis and Reduced Mortality With Treatment. / Fibrotic Liver Disease Consortium Investigators.

In: Clinical Gastroenterology and Hepatology, Vol. 16, No. 8, 01.08.2018, p. 1342-1350.e1.

Research output: Contribution to journalArticle

Fibrotic Liver Disease Consortium Investigators. / Increasing Prevalence of Primary Biliary Cholangitis and Reduced Mortality With Treatment. In: Clinical Gastroenterology and Hepatology. 2018 ; Vol. 16, No. 8. pp. 1342-1350.e1.
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abstract = "Background & Aims: There are few data from longitudinal studies of trends in primary biliary cholangitis (PBC) among patients under routine clinical care in the United States. We collected data from the Fibrotic Liver Disease consortium to investigate changes in the incidence and prevalence of PBC and the effects of patient demographics, clinical features, and treatment on mortality. Methods: We collected demographic and clinical data for the general patient population as well as PBC patients receiving care from 11 health systems in different regions of the United States (Northeast, Midwest, Northwest, and South) from January 1, 2003, through December 31, 2014. Annual percentage changes in PBC prevalence and incidence were estimated using join-point Poisson regression. Differences based on race, age, and gender were calculated with rate ratios. All-cause mortality was estimated using Cox regression with adjustment for patient characteristics and treatment with ursodeoxycholic acid (UDCA). Propensity scores were used to adjust for treatment selection bias. Analyses were adjusted by geographic regions. Results: In our racially diverse cohort of 3488 patients with PBC (21{\%} Hispanic, 8{\%} African American, 7{\%} Asian American), 70{\%} had ever received UDCA. From 2006 through 2014, the prevalence of PBC increased from 21.7 to 39.2 per 100,000 persons. Adjusted annual percentage changes in prevalence differed among age groups (≤40 y, 41–50 y, 51–60 y, 61–70 y, and >70 y), ranging from 3.0{\%} to 7.5{\%} (P <.05). Incidence did not change significantly during the study period (4.2 vs 4.3 per 100,000 person-years in 2006 and 2014, respectively; P =.98). Ratios of prevalence for women vs men (3.9:1) and incidence for women vs men (3.2:1) were consistent over the study period. Among African Americans, the prevalence of PBC increased from 16.9 to 30.8 per 100,000 during the study period, and annual incidence ranged from 2.6 to 6.6 per 100,000 person-years. In adjusted analyses, an increased level of alkaline phosphatase at baseline was associated with significantly higher mortality (adjusted hazard ratios [aHR], 1.24; 95{\%} CI, 1.04–1.48 for patients with levels 1–2 times the upper limit of normal and aHR, 2.27; 95{\%} CI, 1.88–2.73 for patients with levels more than 3 times the upper limit of normal). UDCA treatment was associated with significantly reduced mortality (aHR, 0.57; 95{\%} CI, 0.52–0.64). Conclusions: In an analysis of data from patients receiving routine clinical care in Fibrotic Liver Disease Consortium health systems, we found that the prevalence of PBC increased from 2004 through 2014, despite steady incidence. Patient demographic and clinical characteristics, as well as UDCA treatment, affected mortality.",
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author = "{Fibrotic Liver Disease Consortium Investigators} and Mei Lu and Yueren Zhou and Haller, {Irina V.} and Romanelli, {Robert J.} and VanWormer, {Jeffrey J.} and Rodriguez, {Carla V.} and Heather Anderson and Boscarino, {Joseph A.} and Schmidt, {Mark A.} and Daida, {Yihe G.} and Amandeep Sahota and Jennifer Vincent and Bowlus, {Christopher L.} and Keith Lindor and Talan Zhang and Sheri Trudeau and Jia Li and Rupp, {Loralee B.} and Gordon, {Stuart C.}",
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TY - JOUR

T1 - Increasing Prevalence of Primary Biliary Cholangitis and Reduced Mortality With Treatment

AU - Fibrotic Liver Disease Consortium Investigators

AU - Lu, Mei

AU - Zhou, Yueren

AU - Haller, Irina V.

AU - Romanelli, Robert J.

AU - VanWormer, Jeffrey J.

AU - Rodriguez, Carla V.

AU - Anderson, Heather

AU - Boscarino, Joseph A.

AU - Schmidt, Mark A.

AU - Daida, Yihe G.

AU - Sahota, Amandeep

AU - Vincent, Jennifer

AU - Bowlus, Christopher L.

AU - Lindor, Keith

AU - Zhang, Talan

AU - Trudeau, Sheri

AU - Li, Jia

AU - Rupp, Loralee B.

AU - Gordon, Stuart C.

PY - 2018/8/1

Y1 - 2018/8/1

N2 - Background & Aims: There are few data from longitudinal studies of trends in primary biliary cholangitis (PBC) among patients under routine clinical care in the United States. We collected data from the Fibrotic Liver Disease consortium to investigate changes in the incidence and prevalence of PBC and the effects of patient demographics, clinical features, and treatment on mortality. Methods: We collected demographic and clinical data for the general patient population as well as PBC patients receiving care from 11 health systems in different regions of the United States (Northeast, Midwest, Northwest, and South) from January 1, 2003, through December 31, 2014. Annual percentage changes in PBC prevalence and incidence were estimated using join-point Poisson regression. Differences based on race, age, and gender were calculated with rate ratios. All-cause mortality was estimated using Cox regression with adjustment for patient characteristics and treatment with ursodeoxycholic acid (UDCA). Propensity scores were used to adjust for treatment selection bias. Analyses were adjusted by geographic regions. Results: In our racially diverse cohort of 3488 patients with PBC (21% Hispanic, 8% African American, 7% Asian American), 70% had ever received UDCA. From 2006 through 2014, the prevalence of PBC increased from 21.7 to 39.2 per 100,000 persons. Adjusted annual percentage changes in prevalence differed among age groups (≤40 y, 41–50 y, 51–60 y, 61–70 y, and >70 y), ranging from 3.0% to 7.5% (P <.05). Incidence did not change significantly during the study period (4.2 vs 4.3 per 100,000 person-years in 2006 and 2014, respectively; P =.98). Ratios of prevalence for women vs men (3.9:1) and incidence for women vs men (3.2:1) were consistent over the study period. Among African Americans, the prevalence of PBC increased from 16.9 to 30.8 per 100,000 during the study period, and annual incidence ranged from 2.6 to 6.6 per 100,000 person-years. In adjusted analyses, an increased level of alkaline phosphatase at baseline was associated with significantly higher mortality (adjusted hazard ratios [aHR], 1.24; 95% CI, 1.04–1.48 for patients with levels 1–2 times the upper limit of normal and aHR, 2.27; 95% CI, 1.88–2.73 for patients with levels more than 3 times the upper limit of normal). UDCA treatment was associated with significantly reduced mortality (aHR, 0.57; 95% CI, 0.52–0.64). Conclusions: In an analysis of data from patients receiving routine clinical care in Fibrotic Liver Disease Consortium health systems, we found that the prevalence of PBC increased from 2004 through 2014, despite steady incidence. Patient demographic and clinical characteristics, as well as UDCA treatment, affected mortality.

AB - Background & Aims: There are few data from longitudinal studies of trends in primary biliary cholangitis (PBC) among patients under routine clinical care in the United States. We collected data from the Fibrotic Liver Disease consortium to investigate changes in the incidence and prevalence of PBC and the effects of patient demographics, clinical features, and treatment on mortality. Methods: We collected demographic and clinical data for the general patient population as well as PBC patients receiving care from 11 health systems in different regions of the United States (Northeast, Midwest, Northwest, and South) from January 1, 2003, through December 31, 2014. Annual percentage changes in PBC prevalence and incidence were estimated using join-point Poisson regression. Differences based on race, age, and gender were calculated with rate ratios. All-cause mortality was estimated using Cox regression with adjustment for patient characteristics and treatment with ursodeoxycholic acid (UDCA). Propensity scores were used to adjust for treatment selection bias. Analyses were adjusted by geographic regions. Results: In our racially diverse cohort of 3488 patients with PBC (21% Hispanic, 8% African American, 7% Asian American), 70% had ever received UDCA. From 2006 through 2014, the prevalence of PBC increased from 21.7 to 39.2 per 100,000 persons. Adjusted annual percentage changes in prevalence differed among age groups (≤40 y, 41–50 y, 51–60 y, 61–70 y, and >70 y), ranging from 3.0% to 7.5% (P <.05). Incidence did not change significantly during the study period (4.2 vs 4.3 per 100,000 person-years in 2006 and 2014, respectively; P =.98). Ratios of prevalence for women vs men (3.9:1) and incidence for women vs men (3.2:1) were consistent over the study period. Among African Americans, the prevalence of PBC increased from 16.9 to 30.8 per 100,000 during the study period, and annual incidence ranged from 2.6 to 6.6 per 100,000 person-years. In adjusted analyses, an increased level of alkaline phosphatase at baseline was associated with significantly higher mortality (adjusted hazard ratios [aHR], 1.24; 95% CI, 1.04–1.48 for patients with levels 1–2 times the upper limit of normal and aHR, 2.27; 95% CI, 1.88–2.73 for patients with levels more than 3 times the upper limit of normal). UDCA treatment was associated with significantly reduced mortality (aHR, 0.57; 95% CI, 0.52–0.64). Conclusions: In an analysis of data from patients receiving routine clinical care in Fibrotic Liver Disease Consortium health systems, we found that the prevalence of PBC increased from 2004 through 2014, despite steady incidence. Patient demographic and clinical characteristics, as well as UDCA treatment, affected mortality.

KW - Autoimmune Disease

KW - Epidemiology

KW - FOLD Consortium

KW - Gender

KW - Racial Disparities

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