Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a term applied to a spectrum of immune-mediated demyelinating neuropathies that are heterogeneous in clinical manifestations and probably in pathogenesis. Although histopathologic studies of CIDP have been complicated by a relapsing course of the inflammatory reaction and its predominance in proximal nerve segments, many clues point to involvement of both cellular and humoral immune factors in the pathogenesis. Uncertainties remain regarding the provoking antigen(s) and location of the initial T cell activation. Breakdown of blood nerve barrier by activated T cells and its cytokines is followed by a local intraneural immune response with recruitment of macrophages and secretion of toxic factors, which cause damage to the myelin and axons. Activated T cells may also induce B cells to produce antibodies against nerve/myelin antigens. This review summarizes our current knowledge of the immunopathogenesis and insight from animal models of CIDP.