Autoimmune hepatitis

Abstract

Autoimmune hepatitis is characterized by autoantibodies, hypergammaglobulinemia, interface hepatitis on histologic examination, and the absence of a definable etiologic agent or alternative cause. This chapter describes autoimmune hepatitis and its various clinical phenotypes, presents its putative pathogenic pathways, indicates the codified criteria and scoring systems for diagnosis, presents first-line and rescue management strategies, and reviews immediate and long-term outcomes. Autoimmune hepatitis is rare (incidence in white Europeans, 1 to 1.9 cases per 100,000 persons per year; point prevalence, 11 to 16.9 cases per 100,000 persons), but it affects all ages and ethnic groups. Its variable clinical presentations (acute, acute severe, asymptomatic, chronic) and diverse clinical features (e.g., few or no autoantibodies, overlapping cholestatic findings) warrant its consideration in all patients with acute and chronic liver disease or graft dysfunction after liver transplantation. Prednisone or prednisolone in combination with azathioprine is the preferred first-line treatment, whereas a higher dose of prednisone or prednisolone alone can be used in azathioprine-intolerant patients. Budesonide with azathioprine is an alternative regimen in selected individuals. Relapse, incomplete response, treatment failure, and drug toxicity are outcomes that require alternative strategies, and mycophenolate mofetil and calcineurin inhibitors have been used for some refractory patients. Graft and patient survivals have been excellent after liver transplantation, but autoimmune hepatitis recurs in 36% to 68% within 5 years.