Anti-CD20 therapy and pauci-immune crescentic glomerulonephritis.

Abstract

Pauci-immune glomerulonephritis represents an important cause of crescentic glomerulonephritis, which may be renal-limited vasculitis or part of systemic vasculitis, i.e., microscopic polyangiitis, granulomatosis with polyangiitis (previously known as Wegner’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (previously known as ChurgeStrauss Syndrome). While the presence of antineutrophil cytoplasmic antibody (ANCA) serves a diagnostic tool and leads to the term “ANCA-associated vasculitis,” these autoantibodies are also involved in the pathogenesis. Moreover, there are reports of a positive correlation between B cell activation status and disease activity and the central role of B cells is further supported by reports mentioning that the cyclophosphamide effect is mediated through the suppression of B-cell functions. Thus, suppression of antibody production by Bcell depleting agents to reduce ANCA levels becomes appealing. The murine-human chimeric anti-CD20 monoclonal antibody rituximab depletes B cells and suppresses the production of antibodies. Moreover, rituximab affects other B cell-mediated processes, including but not limited to antigen presentation, cytokine production, and B cell:T cell costimulation, and was associated with the expansion of