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Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis

BACKGROUND AND AIM: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. PSC is frequently associated with inflammatory bowel disease (IBD). Nodular regenerative hyperplasia (NRH) can occur...

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Autores principales: Ozturk, Nazli Begum, Fiel, Maria Isabel, Schiano, Thomas D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wiley Publishing Asia Pty Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9446399/
https://www.ncbi.nlm.nih.gov/pubmed/36091322
http://dx.doi.org/10.1002/jgh3.12795
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author Ozturk, Nazli Begum
Fiel, Maria Isabel
Schiano, Thomas D.
author_facet Ozturk, Nazli Begum
Fiel, Maria Isabel
Schiano, Thomas D.
author_sort Ozturk, Nazli Begum
collection PubMed
description BACKGROUND AND AIM: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. PSC is frequently associated with inflammatory bowel disease (IBD). Nodular regenerative hyperplasia (NRH) can occur in IBD with the use or even in the absence of thiopurine treatment. We aimed to study the significance of the presence of NRH and obliterative portal venopathy (OPV), both causes of non‐cirrhotic portal hypertension (NCPH), in patients having PSC. METHODS: Patients with PSC and concurrent NRH on liver biopsy were identified from the digital pathology database covering the period 2003–2019. Evaluation of liver biopsy and the original diagnoses were confirmed on review based on standard histological features diagnostic for NRH and OPV. Clinical and laboratory data were obtained from electronic medical records. RESULTS: Thirty‐one patients (21 male, 10 female; median age at biopsy 40.1 years) were included in the study. Twelve (38.7%) patients had OPV in addition to NRH on the liver biopsy. Nineteen (61.2%) patients had IBD including 11 with Crohn's disease (CD), 7 with ulcerative colitis (UC), and 1 with indeterminate colitis. Thirteen (41.9%) patients had evidence of portal hypertension, 10 (32.2%) with esophageal varices, 4 (12.9%) with history of variceal bleeding, 6 (19.3%) with ascites, and 14 (12.9%) with splenomegaly. Eleven (35.4%) patients had a cirrhotic‐appearing liver on imaging. Twelve (38.7%) patients had a history of prior or current thiopurine use. CONCLUSIONS: The current study suggests that NRH with or without OPV independently occurs in patients having PSC and may lead to NCPH, even in the absence of concurrent IBD and/or thiopurine therapy.
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spelling pubmed-94463992022-09-09 Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis Ozturk, Nazli Begum Fiel, Maria Isabel Schiano, Thomas D. JGH Open Original Articles BACKGROUND AND AIM: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. PSC is frequently associated with inflammatory bowel disease (IBD). Nodular regenerative hyperplasia (NRH) can occur in IBD with the use or even in the absence of thiopurine treatment. We aimed to study the significance of the presence of NRH and obliterative portal venopathy (OPV), both causes of non‐cirrhotic portal hypertension (NCPH), in patients having PSC. METHODS: Patients with PSC and concurrent NRH on liver biopsy were identified from the digital pathology database covering the period 2003–2019. Evaluation of liver biopsy and the original diagnoses were confirmed on review based on standard histological features diagnostic for NRH and OPV. Clinical and laboratory data were obtained from electronic medical records. RESULTS: Thirty‐one patients (21 male, 10 female; median age at biopsy 40.1 years) were included in the study. Twelve (38.7%) patients had OPV in addition to NRH on the liver biopsy. Nineteen (61.2%) patients had IBD including 11 with Crohn's disease (CD), 7 with ulcerative colitis (UC), and 1 with indeterminate colitis. Thirteen (41.9%) patients had evidence of portal hypertension, 10 (32.2%) with esophageal varices, 4 (12.9%) with history of variceal bleeding, 6 (19.3%) with ascites, and 14 (12.9%) with splenomegaly. Eleven (35.4%) patients had a cirrhotic‐appearing liver on imaging. Twelve (38.7%) patients had a history of prior or current thiopurine use. CONCLUSIONS: The current study suggests that NRH with or without OPV independently occurs in patients having PSC and may lead to NCPH, even in the absence of concurrent IBD and/or thiopurine therapy. Wiley Publishing Asia Pty Ltd 2022-07-19 /pmc/articles/PMC9446399/ /pubmed/36091322 http://dx.doi.org/10.1002/jgh3.12795 Text en © 2022 The Authors. JGH Open published by Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Original Articles
Ozturk, Nazli Begum
Fiel, Maria Isabel
Schiano, Thomas D.
Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis
title Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis
title_full Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis
title_fullStr Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis
title_full_unstemmed Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis
title_short Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis
title_sort identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9446399/
https://www.ncbi.nlm.nih.gov/pubmed/36091322
http://dx.doi.org/10.1002/jgh3.12795
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