Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome

Carey, Elizabeth J.; Somaratne, Krishni; Rakela, Jorge

doi:10.4067/s0034-98872011001100015

Cited by 31 publications

(15 citation statements)

References 15 publications

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“…A middle-aged woman with AIH-PBC overlap syndrome entered remission using prednisone and azathioprine [200]. She appeared to relapse on therapy, but did not respond to intensified prednisone, azathioprine or MMF.…”

Section: Rituximabmentioning

confidence: 95%

Autoimmune hepatitis

Sahebjam

Vierling

2015

Front. Med.

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Autoimmune hepatitis is a chronic liver disease putatively caused by loss of tolerance to hepatocyte-specific autoantigens. It is characterized by female predilection, elevated aminotransferase levels, autoantibodies, increased γ-globulin or IgG levels and biopsy evidence of interface hepatitis. It is currently divided into types 1 and 2, based on expression of autoantibodies. Autoantigenic epitopes have been identified only for the less frequent type 2. Although autoimmune hepatitis occurs in childhood, this review focuses on disease in adults. In the absence of pathognomonic biomarkers, diagnosis requires consideration of clinical, biochemical, serological and histological features, which have been codified into validated diagnostic scoring systems. Since many features also occur in other chronic liver diseases, these scoring systems aid evaluation of the differential diagnosis. New practice guidelines have redefined criteria for remission to include complete biochemical and histological normalization on immunosuppressive therapy. Immunosuppression is most often successful using prednisone or prednisolone and azathioprine; however, the combination of budesonide and azathioprine for non-cirrhotic patients offers distinct advantages. Patients failing standard immunosuppression are candidates for alternative immunosuppressive regimens, yet none of the options has been studied in a randomized, controlled trial. Overlap syndromes with either primary sclerosing cholangitis or primary biliary cirrhosis occur in a minority. Liver transplantation represents a life-saving option for patients presenting with acute liver failure, severely decompensated cirrhosis or hepatocellular carcinoma. Transplant recipients are at risk for recurrent autoimmune hepatitis in the allograft, and de novo disease may occur in patients transplanted for other indications. Patients transplanted for AIH are also at risk for recurrent or de novo inflammatory bowel disease. Progress in our understanding of the immunopathogenesis should lead to identification of specific diagnostic and prognostic biomarkers and new therapeutic strategies.

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Section: Rituximabmentioning

confidence: 95%

Autoimmune hepatitis

Sahebjam

Vierling

2015

Front. Med.

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“…A later report of a patient with concurrent diagnoses of B cell lymphoma and steroid resistant AIH/PBC overlap syndrome showed that a total of a 12-week treatment with rituximab resulted in clinical, biochemical and histological remission of the liver disease [81]. Rituximab has also been reported as an effective treatment of AIH in patients with concomitant idiopatic thrombocytopaenic purpura [82], cryoglobulinaemic glomerulonephritis [83], or Evans syndrome [84]. In a phase 1 study, 6 patients with isolated AIH refractory to standard treatment, were treated with rituximab (1000 mg at days 1 and 15) [85].…”

Section: Rituximabmentioning

confidence: 95%

Autoimmune hepatitis: Contrasts and comparisons in children and adults – A comprehensive review

Floreani

Liberal

Vergani

et al. 2013

Journal of Autoimmunity

105

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“…117,118 These presumptions have been supported by the successful treatment of autoimmune hepatitis and idiopathic thrombocytopaenic purpura 119 ; autoimmune hepatitis and cryoglobulinemic glomerulonephritis 120 ; autoimmune hepatitis and previous B cell lymphoma 121 ; and autoimmune hepatitis and Evans syndrome (autoimmune haemolytic anaemia and idiopathic thrombocytopaenic purpura). 122 A recent study has extended treatment with rituximab to patients without co-existent B-cell disease. In an abstract describing 6 patients with autoimmune hepatitis intolerant of or refractory to therapy with prednisone and azathioprine, rituximab administered intravenously (1000 mg at days 1 and 15) was associated with biochemical resolution and improvement of IgG and c-globulin levels with no serious adverse events.…”

Section: -82mentioning

confidence: 99%

Systematic review: managing suboptimal treatment responses in autoimmune hepatitis with conventional and nonstandard drugs

Selvarajah

Montano‐Loza

Czaja

2012

Aliment Pharmacol Ther

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Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome

Abstract: A 44-year-old woman was found to have elevated aminotransferases, twice the upper limit of normal. Liver biopsy demonstrated a mixed infl ammatory process suggestive of both primary biliary cirrhosis and autoimmune hepatitis (AIH

Cited by 31 publications

References 15 publications

Autoimmune hepatitis

Autoimmune hepatitis

Autoimmune hepatitis: Contrasts and comparisons in children and adults – A comprehensive review

Systematic review: managing suboptimal treatment responses in autoimmune hepatitis with conventional and nonstandard drugs

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