Autoimmune cholangitis associated to IgG4 related sclerosing disease

The IgG4-related sclerosing disease is characterized by the presence of plasmatic IgG4 positive cells and Tlymphocytes infiltration in different organs. We herein report a case of cholest asis due to autoimmune cholangitis associated to IgG4 disease. A 40-year-old woman with a history of pruritus, a...

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Main Authors: Bernardo Frider, Andrés Bruno, Marcelo Zylberman, Alejandro Oría, Marcelo Amante
Format: Article
Language:English
Published: Sociedad Argentina de Gastroenterología 2011-01-01
Series:Acta Gastroenterológica Latinoamericana
Online Access:http://www.redalyc.org/articulo.oa?id=199317366012
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author Bernardo Frider
Andrés Bruno
Marcelo Zylberman
Alejandro Oría
Marcelo Amante
author_facet Bernardo Frider
Andrés Bruno
Marcelo Zylberman
Alejandro Oría
Marcelo Amante
author_sort Bernardo Frider
collection DOAJ
description The IgG4-related sclerosing disease is characterized by the presence of plasmatic IgG4 positive cells and Tlymphocytes infiltration in different organs. We herein report a case of cholest asis due to autoimmune cholangitis associated to IgG4 disease. A 40-year-old woman with a history of pruritus, anosmia, Sjögren's syndrome and diabetes, was referred for a pancreatic tumor. Alkaline phosphatase was 24-fold upper limit of normal (ULN), gamma-glutamyl transpeptidase 21-fold ULN, aspartate aminotransferase 3-fold ULN, alanine aminotransferase 2-fold ULN, cholesterol 408 mg/dL, bilirubin normal, gamma-globulin 3.92 g/dL, IgG4 4.6 g/L, antinuclear antibody positive (1/320), and antimitochondrial antibodies negative. Ultrasound scan (US) showed a mass in the pancreatic head and thickening of the gallbladder and the bile duct walls. Dilation and strictures of the main pancreatic duct and intrahepatic bile ducts were detected by MR cholangiopancreatography. Liver biopsy showed chronic inflammatory lesions, ductal damage (autoimmune cholangitis) (METAVIR A2, F2) and IgG4 bearing plasmatic cells. A cervical lymph node showed IgG4 bearing plasmatic cells. After 2 weeks of treatment with meprednisone, ursodeoxycholic acid and insulin, pruritus and anosmia disappeared. After eleven months of treatment imaging studies showed disappearance of the pancreatic tumor, atrophy of the body and the pancreatic tail and normal biochemical parameters, except for alkaline phosphatase 2-fold ULN. The final diagnosis of our patient was autoimmune hepatitis with cholangitis associated to IgG4 systemic disease.
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spelling doaj.art-ee9752d0b9764d3fa4a96691e92ebb9e2023-01-02T06:37:53ZengSociedad Argentina de GastroenterologíaActa Gastroenterológica Latinoamericana0300-90332011-01-014115559Autoimmune cholangitis associated to IgG4 related sclerosing diseaseBernardo FriderAndrés BrunoMarcelo ZylbermanAlejandro OríaMarcelo AmanteThe IgG4-related sclerosing disease is characterized by the presence of plasmatic IgG4 positive cells and Tlymphocytes infiltration in different organs. We herein report a case of cholest asis due to autoimmune cholangitis associated to IgG4 disease. A 40-year-old woman with a history of pruritus, anosmia, Sjögren's syndrome and diabetes, was referred for a pancreatic tumor. Alkaline phosphatase was 24-fold upper limit of normal (ULN), gamma-glutamyl transpeptidase 21-fold ULN, aspartate aminotransferase 3-fold ULN, alanine aminotransferase 2-fold ULN, cholesterol 408 mg/dL, bilirubin normal, gamma-globulin 3.92 g/dL, IgG4 4.6 g/L, antinuclear antibody positive (1/320), and antimitochondrial antibodies negative. Ultrasound scan (US) showed a mass in the pancreatic head and thickening of the gallbladder and the bile duct walls. Dilation and strictures of the main pancreatic duct and intrahepatic bile ducts were detected by MR cholangiopancreatography. Liver biopsy showed chronic inflammatory lesions, ductal damage (autoimmune cholangitis) (METAVIR A2, F2) and IgG4 bearing plasmatic cells. A cervical lymph node showed IgG4 bearing plasmatic cells. After 2 weeks of treatment with meprednisone, ursodeoxycholic acid and insulin, pruritus and anosmia disappeared. After eleven months of treatment imaging studies showed disappearance of the pancreatic tumor, atrophy of the body and the pancreatic tail and normal biochemical parameters, except for alkaline phosphatase 2-fold ULN. The final diagnosis of our patient was autoimmune hepatitis with cholangitis associated to IgG4 systemic disease.http://www.redalyc.org/articulo.oa?id=199317366012
spellingShingle Bernardo Frider
Andrés Bruno
Marcelo Zylberman
Alejandro Oría
Marcelo Amante
Autoimmune cholangitis associated to IgG4 related sclerosing disease
Acta Gastroenterológica Latinoamericana
title Autoimmune cholangitis associated to IgG4 related sclerosing disease
title_full Autoimmune cholangitis associated to IgG4 related sclerosing disease
title_fullStr Autoimmune cholangitis associated to IgG4 related sclerosing disease
title_full_unstemmed Autoimmune cholangitis associated to IgG4 related sclerosing disease
title_short Autoimmune cholangitis associated to IgG4 related sclerosing disease
title_sort autoimmune cholangitis associated to igg4 related sclerosing disease
url http://www.redalyc.org/articulo.oa?id=199317366012
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AT alejandrooria autoimmunecholangitisassociatedtoigg4relatedsclerosingdisease
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