2268 Rituximab for the Treatment of Refractory Autoimmune Hepatitis

Abstract

INTRODUCTION: Autoimmune hepatitis can be refractory to steroids and traditional immunosuppressant agents especially in the setting of coexisting autoimmune conditions. We describe a patient with autoimmune hepatitis and Neuromyelitis Optica with elevated liver enzymes who was refractory to the traditional immunosuppressant management, but responded to therapy with Rituximab. CASE DESCRIPTION/METHODS: Our patient is a 57-year-old male who was diagnosed with Autoimmune hepatitis after he presented with elevated liver enzymes. Just prior to being evaluated for autoimmune hepatitis, he was diagnosed with Neuromyelitis Optica after extensive investigation when he presented with recurrent symptoms of diffuse paresthesia and muscle weakness distributed all over the body. His liver enzymes at the time of presentation were ALT (1688), AST (656), ALP (193), Bilirubin (1). Autoimmune work up was positive for NMO IgG antibody and ANA, but negative for AMA and ASMA. Liver biopsy was consistent with autoimmune hepatitis showing interface hepatitis with necroinflammatory activity and presence of plasma cells with lobular disarray. Work up for other chronic liver diseases was negative. He initially improved with pulse dose of IV steroids and was later transitioned to maintenance therapy with oral prednisone and Imuran. But, he continued to have moderately elevated liver enzymes with relapses of both autoimmune hepatitis and neurological symptoms on combination of prednisone and Imuran, and also experienced adverse effects from Imuran. He was later transitioned from Imuran to Cellcept without significant improvement. He was finally started on Rituximab infusion due to severe neurologic symptoms with complete normalization of liver enzymes and remission of Neurological manifestations. DISCUSSION: Rituximab is an anti CD 20 monoclonal antibody that depletes B cells. It has been shown to improve many autoimmune conditions and should be considered in refractory cases of autoimmune hepatitis especially in the setting of coexisting autoimmune conditions. Although prior case reports and case series have demonstrated the efficacy and safety of Rituximab in difficult to treat autoimmune hepatitis, it is not currently FDA approved for this purpose and payers will usually deny coverage. Prospective clinical trials of Rituximab in refractory autoimmune hepatitis should be considered for further validation.