Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome

A 44-year-old woman was found to have elevated aminotransferases, twice the upper limit of normal. Liver biopsy demonstrated a mixed inflammatory process suggestive of both primary biliary cirrhosis and autoimmune hepatitis (AIH). Prednisone and azathioprine were started, with normalization of aminotransferases. Six months later, she returned with worsening pruritus and re-evaluation demonstrated probable reactivation of AIH with acute elevation of liver injury tests. Repeat liver biopsy was suggestive of a flare of AIH which did not respond to prednisone, azathioprine, or mycophenolate mofetil. One month later the patient was hospitalized for sudden onset of anemia and thrombocytopenia, suggestive of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura consistent with Evans syndrome. Rituximab was initiated and mycophenolate mofetil discontinued. After one infusion of rituximab, liver injury tests significantly improved. Within four weeks of rituximab infusion (4 doses) the patient's Evans syndrome completely resolved with normal hemoglobin and platelet levels; aminotransferases also significantly improved to less than twice the upper limit of normal.

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Main Authors: Carey,Elizabeth J, Somaratne,Krishni, Rakela,Jorge
Format: Digital revista
Language:English
Published: Sociedad Médica de Santiago 2011
Online Access:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011001100015
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spelling oai:scielo:S0034-988720110011000152012-02-08Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndromeCarey,Elizabeth JSomaratne,KrishniRakela,Jorge Anemia hemolytic autoimmune Evans syndrome Hepatitis autoimmune Rituximab A 44-year-old woman was found to have elevated aminotransferases, twice the upper limit of normal. Liver biopsy demonstrated a mixed inflammatory process suggestive of both primary biliary cirrhosis and autoimmune hepatitis (AIH). Prednisone and azathioprine were started, with normalization of aminotransferases. Six months later, she returned with worsening pruritus and re-evaluation demonstrated probable reactivation of AIH with acute elevation of liver injury tests. Repeat liver biopsy was suggestive of a flare of AIH which did not respond to prednisone, azathioprine, or mycophenolate mofetil. One month later the patient was hospitalized for sudden onset of anemia and thrombocytopenia, suggestive of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura consistent with Evans syndrome. Rituximab was initiated and mycophenolate mofetil discontinued. After one infusion of rituximab, liver injury tests significantly improved. Within four weeks of rituximab infusion (4 doses) the patient's Evans syndrome completely resolved with normal hemoglobin and platelet levels; aminotransferases also significantly improved to less than twice the upper limit of normal.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.139 n.11 20112011-11-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011001100015en10.4067/S0034-98872011001100015
institution SCIELO
collection OJS
country Chile
countrycode CL
component Revista
access En linea
databasecode rev-scielo-cl
tag revista
region America del Sur
libraryname SciELO
language English
format Digital
author Carey,Elizabeth J
Somaratne,Krishni
Rakela,Jorge
spellingShingle Carey,Elizabeth J
Somaratne,Krishni
Rakela,Jorge
Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome
author_facet Carey,Elizabeth J
Somaratne,Krishni
Rakela,Jorge
author_sort Carey,Elizabeth J
title Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome
title_short Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome
title_full Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome
title_fullStr Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome
title_full_unstemmed Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome
title_sort successful rituximab therapy in refractory autoimmune hepatitis and evans syndrome
description A 44-year-old woman was found to have elevated aminotransferases, twice the upper limit of normal. Liver biopsy demonstrated a mixed inflammatory process suggestive of both primary biliary cirrhosis and autoimmune hepatitis (AIH). Prednisone and azathioprine were started, with normalization of aminotransferases. Six months later, she returned with worsening pruritus and re-evaluation demonstrated probable reactivation of AIH with acute elevation of liver injury tests. Repeat liver biopsy was suggestive of a flare of AIH which did not respond to prednisone, azathioprine, or mycophenolate mofetil. One month later the patient was hospitalized for sudden onset of anemia and thrombocytopenia, suggestive of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura consistent with Evans syndrome. Rituximab was initiated and mycophenolate mofetil discontinued. After one infusion of rituximab, liver injury tests significantly improved. Within four weeks of rituximab infusion (4 doses) the patient's Evans syndrome completely resolved with normal hemoglobin and platelet levels; aminotransferases also significantly improved to less than twice the upper limit of normal.
publisher Sociedad Médica de Santiago
publishDate 2011
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011001100015
work_keys_str_mv AT careyelizabethj successfulrituximabtherapyinrefractoryautoimmunehepatitisandevanssyndrome
AT somaratnekrishni successfulrituximabtherapyinrefractoryautoimmunehepatitisandevanssyndrome
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