The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration

There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long‐term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a surviva...

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Published inHepatology (Baltimore, Md.) Vol. 66; no. 2; pp. 518 - 527
Main Authors Deneau, Mark R., El‐Matary, Wael, Valentino, Pamela L., Abdou, Reham, Alqoaer, Khaled, Amin, Mansi, Amir, Achiya Z., Auth, Marcus, Bazerbachi, Fateh, Broderick, Annemarie, Chan, Albert, Cotter, Jillian, Doan, Sylvia, El‐Youssef, Mounif, Ferrari, Federica, Furuya, Katryn N., Gottrand, Madeleine, Gottrand, Frederic, Gupta, Nitika, Homan, Matjaz, Kamath, Binita M., Kim, Kyung Mo, Kolho, Kaija‐Leena, Konidari, Anastasia, Koot, Bart, Iorio, Raffaele, Ledder, Oren, Mack, Cara, Martinez, Mercedes, Miloh, Tamir, Mohan, Parvathi, O'Cathain, Niamh, Papadopoulou, Alexandra, Ricciuto, Amanda, Saubermann, Lawrence, Sathya, Pushpa, Shteyer, Eyal, Smolka, Vratislav, Tanaka, Atushi, Varier, Raghu, Venkat, Veena, Vitola, Bernadette, Vos, Miriam B., Woynarowski, Marek, Yap, Jason, Jensen, M. Kyle
Format Journal Article
LanguageEnglish
Published United States Wiley Subscription Services, Inc 01.08.2017
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Abstract There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long‐term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death. We analyzed patients grouped by disease phenotype and laboratory studies at diagnosis to identify objective predictors of long‐term outcome. We identified 781 patients, median age 12 years, with 4,277 person‐years of follow‐up; 33% with autoimmune hepatitis, 76% with inflammatory bowel disease, and 13% with small duct PSC. Portal hypertensive and biliary complications developed in 38% and 25%, respectively, after 10 years of disease. Once these complications developed, median survival with native liver was 2.8 and 3.5 years, respectively. Cholangiocarcinoma occurred in 1%. Overall event‐free survival was 70% at 5 years and 53% at 10 years. Patient groups with the most elevated total bilirubin, gamma‐glutamyltransferase, and aspartate aminotransferase‐to‐platelet ratio index at diagnosis had the worst outcomes. In multivariate analysis PSC–inflammatory bowel disease and small duct phenotypes were associated with favorable prognosis (hazard ratios 0.6, 95% confidence interval 0.5‐0.9, and 0.7, 95% confidence interval 0.5‐0.96, respectively). Age, gender, and autoimmune hepatitis overlap did not impact long‐term outcome. Conclusion: PSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma‐glutamyltransferase, and aspartate aminotransferase‐to‐platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC–inflammatory bowel disease are more favorable disease phenotypes. (Hepatology 2017;66:518–527).
AbstractList There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long‐term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death. We analyzed patients grouped by disease phenotype and laboratory studies at diagnosis to identify objective predictors of long‐term outcome. We identified 781 patients, median age 12 years, with 4,277 person‐years of follow‐up; 33% with autoimmune hepatitis, 76% with inflammatory bowel disease, and 13% with small duct PSC. Portal hypertensive and biliary complications developed in 38% and 25%, respectively, after 10 years of disease. Once these complications developed, median survival with native liver was 2.8 and 3.5 years, respectively. Cholangiocarcinoma occurred in 1%. Overall event‐free survival was 70% at 5 years and 53% at 10 years. Patient groups with the most elevated total bilirubin, gamma‐glutamyltransferase, and aspartate aminotransferase‐to‐platelet ratio index at diagnosis had the worst outcomes. In multivariate analysis PSC–inflammatory bowel disease and small duct phenotypes were associated with favorable prognosis (hazard ratios 0.6, 95% confidence interval 0.5‐0.9, and 0.7, 95% confidence interval 0.5‐0.96, respectively). Age, gender, and autoimmune hepatitis overlap did not impact long‐term outcome. Conclusion: PSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma‐glutamyltransferase, and aspartate aminotransferase‐to‐platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC–inflammatory bowel disease are more favorable disease phenotypes. (Hepatology 2017;66:518–527).
There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death. We analyzed patients grouped by disease phenotype and laboratory studies at diagnosis to identify objective predictors of long-term outcome. We identified 781 patients, median age 12 years, with 4,277 person-years of follow-up; 33% with autoimmune hepatitis, 76% with inflammatory bowel disease, and 13% with small duct PSC. Portal hypertensive and biliary complications developed in 38% and 25%, respectively, after 10 years of disease. Once these complications developed, median survival with native liver was 2.8 and 3.5 years, respectively. Cholangiocarcinoma occurred in 1%. Overall event-free survival was 70% at 5 years and 53% at 10 years. Patient groups with the most elevated total bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis had the worst outcomes. In multivariate analysis PSC-inflammatory bowel disease and small duct phenotypes were associated with favorable prognosis (hazard ratios 0.6, 95% confidence interval 0.5-0.9, and 0.7, 95% confidence interval 0.5-0.96, respectively). Age, gender, and autoimmune hepatitis overlap did not impact long-term outcome. PSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC-inflammatory bowel disease are more favorable disease phenotypes. (Hepatology 2017;66:518-527).
There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death. We analyzed patients grouped by disease phenotype and laboratory studies at diagnosis to identify objective predictors of long-term outcome. We identified 781 patients, median age 12 years, with 4,277 person-years of follow-up; 33% with autoimmune hepatitis, 76% with inflammatory bowel disease, and 13% with small duct PSC. Portal hypertensive and biliary complications developed in 38% and 25%, respectively, after 10 years of disease. Once these complications developed, median survival with native liver was 2.8 and 3.5 years, respectively. Cholangiocarcinoma occurred in 1%. Overall event-free survival was 70% at 5 years and 53% at 10 years. Patient groups with the most elevated total bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis had the worst outcomes. In multivariate analysis PSC-inflammatory bowel disease and small duct phenotypes were associated with favorable prognosis (hazard ratios 0.6, 95% confidence interval 0.5-0.9, and 0.7, 95% confidence interval 0.5-0.96, respectively). Age, gender, and autoimmune hepatitis overlap did not impact long-term outcome.CONCLUSIONPSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma-glutamyltransferase, and aspartate aminotransferase-to-platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC-inflammatory bowel disease are more favorable disease phenotypes. (Hepatology 2017;66:518-527).
There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long‐term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death. We analyzed patients grouped by disease phenotype and laboratory studies at diagnosis to identify objective predictors of long‐term outcome. We identified 781 patients, median age 12 years, with 4,277 person‐years of follow‐up; 33% with autoimmune hepatitis, 76% with inflammatory bowel disease, and 13% with small duct PSC. Portal hypertensive and biliary complications developed in 38% and 25%, respectively, after 10 years of disease. Once these complications developed, median survival with native liver was 2.8 and 3.5 years, respectively. Cholangiocarcinoma occurred in 1%. Overall event‐free survival was 70% at 5 years and 53% at 10 years. Patient groups with the most elevated total bilirubin, gamma‐glutamyltransferase, and aspartate aminotransferase‐to‐platelet ratio index at diagnosis had the worst outcomes. In multivariate analysis PSC–inflammatory bowel disease and small duct phenotypes were associated with favorable prognosis (hazard ratios 0.6, 95% confidence interval 0.5‐0.9, and 0.7, 95% confidence interval 0.5‐0.96, respectively). Age, gender, and autoimmune hepatitis overlap did not impact long‐term outcome. Conclusion : PSC has a chronic, progressive course in children, and nearly half of patients develop an adverse liver outcome after 10 years of disease; elevations in bilirubin, gamma‐glutamyltransferase, and aspartate aminotransferase‐to‐platelet ratio index at diagnosis can identify patients at highest risk; small duct PSC and PSC–inflammatory bowel disease are more favorable disease phenotypes. (H epatology 2017;66:518–527).
Author Vitola, Bernadette
Valentino, Pamela L.
Konidari, Anastasia
Bazerbachi, Fateh
Homan, Matjaz
El‐Youssef, Mounif
Ferrari, Federica
Papadopoulou, Alexandra
Amin, Mansi
El‐Matary, Wael
Iorio, Raffaele
Miloh, Tamir
Furuya, Katryn N.
Vos, Miriam B.
Ledder, Oren
Ricciuto, Amanda
Alqoaer, Khaled
Shteyer, Eyal
Woynarowski, Marek
Auth, Marcus
Chan, Albert
Kim, Kyung Mo
Saubermann, Lawrence
Gottrand, Madeleine
Cotter, Jillian
Kolho, Kaija‐Leena
Koot, Bart
Deneau, Mark R.
Martinez, Mercedes
Mohan, Parvathi
Smolka, Vratislav
O'Cathain, Niamh
Abdou, Reham
Varier, Raghu
Broderick, Annemarie
Kamath, Binita M.
Mack, Cara
Gupta, Nitika
Tanaka, Atushi
Gottrand, Frederic
Sathya, Pushpa
Venkat, Veena
Jensen, M. Kyle
Yap, Jason
Amir, Achiya Z.
Doan, Sylvia
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  organization: University of Utah
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  givenname: Federica
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/28390159$$D View this record in MEDLINE/PubMed
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Notes See Editorial on Page
Potential conflict of interest: Dr. Mohan received grants from Gilead. Dr. Auth received grants from AbbVie and Nutricia. Dr. Kamath consults for Retrophin. Dr. Miloh is on the speakers' bureau for Alexion. Dr. Vos consults and received grants from Shire. She consults for Intercept, Target, and Allergan. She received grants from Resonance.
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Snippet There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and...
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SubjectTerms Analysis of Variance
Aspartate aminotransferase
Bilirubin
Biopsy, Needle
Child
Children
Cholangiocarcinoma
Cholangitis
Cholangitis, Sclerosing - mortality
Cholangitis, Sclerosing - pathology
Cholangitis, Sclerosing - surgery
Cohort Studies
Confidence intervals
Disease Progression
Disease-Free Survival
Female
Follow-Up Studies
Hepatitis
Hepatology
Humans
Hypertension
Immunohistochemistry
Inflammatory bowel disease
Inflammatory bowel diseases
Internationality
Intestine
Japan
Liver
Liver Function Tests
Liver transplantation
Liver Transplantation - methods
Liver Transplantation - mortality
Male
Medical prognosis
Multivariate Analysis
Pediatrics
Predictive Value of Tests
Proportional Hazards Models
Retrospective Studies
Risk Assessment
Severity of Illness Index
Survival
Survival Analysis
Transplantation
γ-Glutamyltransferase
Title The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration
URI https://onlinelibrary.wiley.com/doi/abs/10.1002%2Fhep.29204
https://www.ncbi.nlm.nih.gov/pubmed/28390159
https://www.proquest.com/docview/1920069016
https://search.proquest.com/docview/1885950980
Volume 66
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