Presence of ectopic germinal center structures in autoimmune hepatitis

Abstract

Autoimmune tissues may contain ectopic germinal centers (EGCs). However, these structures have never been described in the liver of patients suffering from autoimmune hepatitis (AIH). We retrospectively reviewed histological features of 120 definite AIH cases, and found 10 cases harboring markers of EGCs. In these cases, CD21+ follicular dendritic cells were intermixed with CD3+ T and CD20+ B lymphocytes. The latter expressed the GC-specific marker bcl6, and some were proliferative as assessed by Ki67 expression. Antibody-secreting cells (ASCs) defined by expression of the mum-1 transcription factor and presence of cytoplasmic IgMs were usually present in the periphery of these structures, but some were also present within the EGCs. Notably, some ASCs were IgG-switched. Common treatment applied to AIH patients achieved biochemical normalization as efficiently as in patients without EGCs. In the present study, we provide the proof for the occurrence of functional EGCs enabling differentiation of B cells into ASCs and occurrence of immunoglobulin switch in AIH livers.