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Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community

Overlap syndrome (OS) is a term that comprises the presentation of multiple hepatic disease characteristics in the same patient, such as the presence of autoimmune hepatitis (AIH) features in addition to primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC). Standard therapy for...

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Autores principales: Guifarro, Daniel A, De Oliveira-Gomes, Diana, Beas, Renato, Yibirin-Wakim, Marcel J, Montalvan-Sanchez, Eleazar E
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10099648/
https://www.ncbi.nlm.nih.gov/pubmed/37065404
http://dx.doi.org/10.7759/cureus.36126
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author Guifarro, Daniel A
De Oliveira-Gomes, Diana
Beas, Renato
Yibirin-Wakim, Marcel J
Montalvan-Sanchez, Eleazar E
author_facet Guifarro, Daniel A
De Oliveira-Gomes, Diana
Beas, Renato
Yibirin-Wakim, Marcel J
Montalvan-Sanchez, Eleazar E
author_sort Guifarro, Daniel A
collection PubMed
description Overlap syndrome (OS) is a term that comprises the presentation of multiple hepatic disease characteristics in the same patient, such as the presence of autoimmune hepatitis (AIH) features in addition to primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC). Standard therapy for AIH is immunosuppression, while ursodeoxycholic acid is the preferred treatment for PBC. Additionally, liver transplantation (LT) may be considered in severe cases. Hispanics have been found to have a higher prevalence of chronic liver disease and develop more complications associated with portal hypertension at the time of listing for LT. Despite being the fastest-growing population in the USA, Hispanics have a higher probability of not receiving an LT due to issues with social determinants of health (SDOH). It has been reported that Hispanics are more likely to be removed from the transplant list. We report a case of a 25-year-old female immigrant from a Latin American developing country who presented with symptoms consistent with worsening liver disease after years of inappropriate workup and late diagnosis due to barriers within the healthcare system. The patient had a history of unresolved jaundice and pruritus and presented with worsening of her previous symptoms and new onset abdominal distention, bilateral leg edema, and telangiectasias. Laboratory and imaging studies confirmed the diagnosis of AIH and primary sclerosing cholangitis (PSC-AIH syndrome). The patient was started on steroids, azathioprine, and ursodeoxycholic acid, showing improvement. Due to her migratory status, she could not receive an appropriate diagnosis and follow up with a single provider or healthcare institution, putting her at increased risk for life-threatening complications. Although medical management is the first step, the probability of future liver transplants exists. The patient is still undergoing liver transplant evaluation and completing a workup since she was found to have an elevated model for end-stage liver disease (MELD) score. Even with the introduction of new scores and policies that aim to reduce disparities in LT, Hispanic patients are still at higher risk of being removed from the waitlist because of death or clinical deterioration compared to non-Hispanics. To this day, Hispanics have the highest percentage of waitlist deaths (20.8%) of all ethnicities and the lowest overall rate for undergoing LT. Understanding and addressing the causes that could contribute to and explain this phenomenon is essential. Increasing awareness of this problem is vital to promote more research on LT disparities.
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spelling pubmed-100996482023-04-14 Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community Guifarro, Daniel A De Oliveira-Gomes, Diana Beas, Renato Yibirin-Wakim, Marcel J Montalvan-Sanchez, Eleazar E Cureus Internal Medicine Overlap syndrome (OS) is a term that comprises the presentation of multiple hepatic disease characteristics in the same patient, such as the presence of autoimmune hepatitis (AIH) features in addition to primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC). Standard therapy for AIH is immunosuppression, while ursodeoxycholic acid is the preferred treatment for PBC. Additionally, liver transplantation (LT) may be considered in severe cases. Hispanics have been found to have a higher prevalence of chronic liver disease and develop more complications associated with portal hypertension at the time of listing for LT. Despite being the fastest-growing population in the USA, Hispanics have a higher probability of not receiving an LT due to issues with social determinants of health (SDOH). It has been reported that Hispanics are more likely to be removed from the transplant list. We report a case of a 25-year-old female immigrant from a Latin American developing country who presented with symptoms consistent with worsening liver disease after years of inappropriate workup and late diagnosis due to barriers within the healthcare system. The patient had a history of unresolved jaundice and pruritus and presented with worsening of her previous symptoms and new onset abdominal distention, bilateral leg edema, and telangiectasias. Laboratory and imaging studies confirmed the diagnosis of AIH and primary sclerosing cholangitis (PSC-AIH syndrome). The patient was started on steroids, azathioprine, and ursodeoxycholic acid, showing improvement. Due to her migratory status, she could not receive an appropriate diagnosis and follow up with a single provider or healthcare institution, putting her at increased risk for life-threatening complications. Although medical management is the first step, the probability of future liver transplants exists. The patient is still undergoing liver transplant evaluation and completing a workup since she was found to have an elevated model for end-stage liver disease (MELD) score. Even with the introduction of new scores and policies that aim to reduce disparities in LT, Hispanic patients are still at higher risk of being removed from the waitlist because of death or clinical deterioration compared to non-Hispanics. To this day, Hispanics have the highest percentage of waitlist deaths (20.8%) of all ethnicities and the lowest overall rate for undergoing LT. Understanding and addressing the causes that could contribute to and explain this phenomenon is essential. Increasing awareness of this problem is vital to promote more research on LT disparities. Cureus 2023-03-14 /pmc/articles/PMC10099648/ /pubmed/37065404 http://dx.doi.org/10.7759/cureus.36126 Text en Copyright © 2023, Guifarro et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Guifarro, Daniel A
De Oliveira-Gomes, Diana
Beas, Renato
Yibirin-Wakim, Marcel J
Montalvan-Sanchez, Eleazar E
Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community
title Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community
title_full Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community
title_fullStr Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community
title_full_unstemmed Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community
title_short Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community
title_sort primary sclerosing cholangitis-autoimmune hepatitis overlap syndrome: significant barriers in liver disease diagnosis and treatment experienced by the latino community
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10099648/
https://www.ncbi.nlm.nih.gov/pubmed/37065404
http://dx.doi.org/10.7759/cureus.36126
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