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Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?

INTRODUCTION: Autoimmune hepatitis is an inflammatory disease with multifactorial ethiopatogenesis, characterized by lympho-monocytic infiltration of liver, presence of serum autoantibodies (ANA, SMA, LKM-1) and high levels of immunoglobulins. Overlap syndromes are defined as the association of auto...

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Autores principales: Pratico, Andrea Domenico, Salafia, Stefania, Barone, Patrizia, La Rosa, Mario, Leonardi, Salvatore
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Kowsar 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3867022/
https://www.ncbi.nlm.nih.gov/pubmed/24358042
http://dx.doi.org/10.5812/hepatmon.14452
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author Pratico, Andrea Domenico
Salafia, Stefania
Barone, Patrizia
La Rosa, Mario
Leonardi, Salvatore
author_facet Pratico, Andrea Domenico
Salafia, Stefania
Barone, Patrizia
La Rosa, Mario
Leonardi, Salvatore
author_sort Pratico, Andrea Domenico
collection PubMed
description INTRODUCTION: Autoimmune hepatitis is an inflammatory disease with multifactorial ethiopatogenesis, characterized by lympho-monocytic infiltration of liver, presence of serum autoantibodies (ANA, SMA, LKM-1) and high levels of immunoglobulins. Overlap syndromes are defined as the association of autoimmune hepatitis with cholestatic diseases such as primary biliary cirrhosis and primary sclerosing cholangitis. The boundaries of these syndromes as distinct pathological entities are still matter of debate and they could be part of a major liver autoimmune disease. Furthermore, cholestatic diseases may present even with atypical features (AMA-negative primary cirrohosis, primary sclerosing cholangitis with normal cholangiography). CASE PRESENTATION: We herein describe a case of a 7 year-old child affected by an overlap syndrome between type 2 autoimmune hepatitis and small duct primary sclerosing cholangitis. Although characterized by a severe onset, the disease showed a good response to treatment with prednisone and azathioprine. CONCLUSIONS: The association of type 2 autoimmune hepatitis and small duct primary cholangitis has been rarely reported in literature and this report adds new data on this still unclear entity.
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spelling pubmed-38670222013-12-19 Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome? Pratico, Andrea Domenico Salafia, Stefania Barone, Patrizia La Rosa, Mario Leonardi, Salvatore Hepat Mon Case Report INTRODUCTION: Autoimmune hepatitis is an inflammatory disease with multifactorial ethiopatogenesis, characterized by lympho-monocytic infiltration of liver, presence of serum autoantibodies (ANA, SMA, LKM-1) and high levels of immunoglobulins. Overlap syndromes are defined as the association of autoimmune hepatitis with cholestatic diseases such as primary biliary cirrhosis and primary sclerosing cholangitis. The boundaries of these syndromes as distinct pathological entities are still matter of debate and they could be part of a major liver autoimmune disease. Furthermore, cholestatic diseases may present even with atypical features (AMA-negative primary cirrohosis, primary sclerosing cholangitis with normal cholangiography). CASE PRESENTATION: We herein describe a case of a 7 year-old child affected by an overlap syndrome between type 2 autoimmune hepatitis and small duct primary sclerosing cholangitis. Although characterized by a severe onset, the disease showed a good response to treatment with prednisone and azathioprine. CONCLUSIONS: The association of type 2 autoimmune hepatitis and small duct primary cholangitis has been rarely reported in literature and this report adds new data on this still unclear entity. Kowsar 2013-12-06 /pmc/articles/PMC3867022/ /pubmed/24358042 http://dx.doi.org/10.5812/hepatmon.14452 Text en Copyright © 2013, Kowsar Corp. http://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 work is properly cited.
spellingShingle Case Report
Pratico, Andrea Domenico
Salafia, Stefania
Barone, Patrizia
La Rosa, Mario
Leonardi, Salvatore
Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?
title Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?
title_full Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?
title_fullStr Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?
title_full_unstemmed Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?
title_short Type II Autoimmune Hepatitis and Small Duct Sclerosing Cholangitis in a Seven Years Old Child: An Overlap Syndrome?
title_sort type ii autoimmune hepatitis and small duct sclerosing cholangitis in a seven years old child: an overlap syndrome?
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3867022/
https://www.ncbi.nlm.nih.gov/pubmed/24358042
http://dx.doi.org/10.5812/hepatmon.14452
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