Targeting the B cell lineage in IgG4-related disease

Abstract

Despite the elevated serum concentration of IgG4 in many patients and the disproportionate number of IgG4+ plasma cells within the affected tissues that constitute essentially a sine qua non of IgG4-related disease, serum IgG4 itself appears to be a reactive phenomenon rather than the primary disease driver. Recent investigations have focused on the interactions between cells of the B cell lineage and a novel CD4+ cytotoxic T cell that is capable of promoting fibrosis.