2022 年 39 巻 4 号 p. 444-446
Immune–mediated neuropathy comprises various peripheral neuropathies caused by immune attack against peripheral nerve axon, myelin or vasa nervosum that nourish all components within peripheral nervous system. Guillain–Barré syndrome, most popular acquired demyelinating neuropathy, are caused by attacks of autoantibodies against antigens existing on peripheral nerve. In contrast, precise mechanism of CIDP (chronic inflammatory demyelinating neuropathy), the other popular acquired demyelinating neuropathy, remain vague including evidence of autoantibody. Recently, autoantibodies against various adhesion molecules distributed on node–paranode regions of peripheral nerve fibers are characterized in some patients with CIDP phenotype. Being their symptoms and therapeutic response different from those of CIDP, these patients are classified as different entity, autoimmune nodo–paranodopathy (ANP). In parallel, better therapeutic options for GBS, CIDP, ANP, as well as vasculitic neuropathy have been rapidly gaining．
In this lecture, recent progress of disease basis, new guide lines, therapy and ongoing trials especially biological drug are reviewed.
