The current state of the problem of seronegative autoimmune hepatitis in children

Abstract

Seronegative autoimmune hepatitis (AIH) is a poorly understood problem in both adult patients and children. The key to the diagnosis of AIH is the presence of circulating autoantibodies, which are not detected in seronegative AIH. There is insufficient data on the contribution of autoantibodies to hepatocyte damage in seronegative AIH. The presence of plasma cells in liver biopsies of patients with seronegative AIH suggests that its pathogenesis involves physiopathological mechanisms similar to those of seropositive AIH. Unlike adults, in whom acute manifestations of the disease are rare, in children’s acute manifestations of seronegative AIH were observed in three quarters of patients. In addition to the absence of autoantibodies, the diagnosis is complicated by the low level of gammaglobulins in the blood in seronegative AIH. In seronegative AIH, hepatitis-associated aplastic anemia often develops. Morphological examination of liver biopsies may reveal infiltration with a predominance of CD8+ T cells. Treatment of seronegative AIH includes immunosuppressive therapy, as for seropositive AIH. The prognosis for seronegative AIH is usually favorable. Although seronegative autoimmune hepatitis is not uncommon, little is known about its diagnosis and treatment.