Autoimmune cholangitis associated to IgG4 related sclerosing disease
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Sociedad Argentina de Gastroenterología
2011
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| _version_ | 1876448291892232192 |
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| author | Bernardo Frider |
| author_facet | Bernardo Frider |
| contents | Autoimmune cholangitis associated to IgG4 related sclerosing disease Bernardo Frider Andrés Bruno Marcelo Zylberman Alejandro Oría Marcelo Amante Medicina IgG4 Cholestasis Autoimmune cholangitis Autoimmune pancreatitis 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. Ultrasoundscan (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. 2011 artículo científico 2469-1119 https://www.redalyc.org/articulo.oa?id=199317366012 es http://www.redalyc.org/revista.oa?id=1993 Acta Gastroenterológica Latinoamericana application/pdf Sociedad Argentina de Gastroenterología Acta Gastroenterológica Latinoamericana (Argentina) Num.1 Vol.41 |
| format | Artículo científico |
| id | redalyc_199317366012 |
| institution | Redalyc |
| language | es |
| publishDate | 2011 |
| publisher | Sociedad Argentina de Gastroenterología |
| spellingShingle | Autoimmune cholangitis associated to IgG4 related sclerosing disease Bernardo Frider Medicina IgG4 Cholestasis Autoimmune cholangitis Autoimmune pancreatitis Autoimmune cholangitis associated to IgG4 related sclerosing disease Bernardo Frider Andrés Bruno Marcelo Zylberman Alejandro Oría Marcelo Amante Medicina IgG4 Cholestasis Autoimmune cholangitis Autoimmune pancreatitis 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. Ultrasoundscan (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. 2011 artículo científico 2469-1119 https://www.redalyc.org/articulo.oa?id=199317366012 es http://www.redalyc.org/revista.oa?id=1993 Acta Gastroenterológica Latinoamericana application/pdf Sociedad Argentina de Gastroenterología Acta Gastroenterológica Latinoamericana (Argentina) Num.1 Vol.41 |
| title | Autoimmune cholangitis associated to IgG4 related sclerosing disease |
| topic | Medicina IgG4 Cholestasis Autoimmune cholangitis Autoimmune pancreatitis |
| url | https://www.redalyc.org/articulo.oa?id=199317366012 |