2016 Volume 33 Issue 2 Pages 168-171
First–line therapies (intravenous immunoglobulin, corticosteroids, and plasmapheresis) of CIDP are already established, but cannot be excessive expectations to the effectiveness of the second–line such as immunosuppressant. Usefulness of the maintenance therapy represented by continuous half–dose IVIg or subcutaneous immunoglobulin (SCIg) has been widely recognized. However, the proper amount of immunoglobulin for maintenance therapy is not clear. In addition, the proper period to begin the withdrawal from the maintenance therapy is not clarified yet. We should find the biomarkers to distinguish the patients who need the maintenance therapy from the “benign” others with monophasic course.
From the pathogenesis of CIDP, autoantibodies that target the molecules (eg. CNTN1, NF155) near the nodes of Ranvier are detected in at least a few percent of whole CIDP patients. These autoantibodies are unique since the autoantibody–positive patients show the similar phenotype including IVIg poor–responsiveness. The usefulness of therapeutics that could remove the antibody such as plasmapheresis, rituximab, and autologous haematopoietic stem cell transplantation (AHSCT) could be expected as a promising treatment. Finally, we should carefully consider those autoantibodies when setting up future clinical trials for CIDP.
