Management of refractory autoimmune hepatitis with rituximab: a case series

TL;DR

Rituximab is a safe and effective treatment option for autoimmune hepatitis patients who do not respond to standard therapies.

Contribution

This case series adds to the limited evidence on rituximab's use in refractory autoimmune hepatitis.

Findings

Rituximab improved transaminases and immunoglobulin G levels in autoimmune hepatitis patients.

Rituximab reduced prednisolone dosage and disease-modifying agents in treated patients.

Rituximab also effectively treated comorbid immune thrombocytopenia in one case.

Abstract

Most patients with autoimmune hepatitis respond to the standard of care of prednisolone in combination with a thiopurine, or a second line of mycophenolate mofetil or tacrolimus. This study aims to add to the reported experience of using rituximab in the small numbers of patients with autoimmune hepatitis who are refractory or intolerant to these treatments.

A retrospective single center case series was performed on six patients who were treated with rituximab for probable or definite, biopsy-proven type 1 autoimmune hepatitis over a 9-year period. They were Caucasian, three males and three females, with an age at autoimmune hepatitis diagnosis ranging from 16 to 52 years old. Three patients had cirrhosis. All six patients had trialed prednisolone, a thiopurine, and also mycophenolate mofetil prior to the rituximab. Indications for rituximab were treatment of an autoimmune hepatitis flare and potential intolerance to the second line therapy. For patients with autoimmune hepatitis flare, rituximab improved transaminases and/or total immunoglobulin G levels, lowered prednisolone dosage, and reduced the number or dose of disease-modifying agents. Four of the six cases required more than one dose of rituximab. Dosage intervals ranged from 1 month to 5 years. In one case, rituximab also effectively treated comorbid immune thrombocytopenia. No complications arose from rituximab treatment.

Rituximab appears safe, and also effective in some patients with autoimmune hepatitis who have been refractory to standard of care. The dose reduction for other disease modifying agents makes rituximab an attractive option for this difficult to treat subset of patients.

The online version contains supplementary material available at 10.1186/s13256-025-05595-3.

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