Autoimmune inflammatory neuropathies: updates in pathogenesis, diagnosis, and treatment.
OBJECTIVE
The present review aims at discussing the recent advances in pathogenesis, diagnosis, and treatment of major subtypes of autoimmune inflammatory neuropathies.


RESULTS
Concerning pathogenesis, further evidence has proved that antibodies to nodal proteins are pathogenic in inflammatory neuropathies. The presence of these antibodies is related to distinctive clinical features. Disruption of blood-nerve barrier mediated by cytokines and chemokines also plays an important role in the pathogenesis. The new terminology of 'nodopathies' describes immune-mediated attack beginning and limited to the nodal region, and this phenomenon can be found in both acute and chronic inflammatory neuropathies. Recent trials comparing intravenous and subcutaneous immunoglobulin confirm that subcutaneous immunoglobulin is not only cost-effective, but also improves patients' satisfaction and quality of life compared to intravenous immunoglobulin. Although immunotherapies are effective in most of the inflammatory neuropathies, accurate predictors and biomarkers of treatment response are lacking. Moreover, some patients do not respond to current immunotherapies and continue to relapse after discontinuation of treatment.


CONCLUSIONS
More studies are required to understand the exact antigenic targets and mechanism in inflammatory neuropathies, so as to develop more novel immunotherapies.
aDepartment of Medicine bDepartment of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore cBrain & Mind Research Institute, The University of Sydney.
Anna Hiu Yi
AH
Wong
Nobuhiro
N
Yuki
D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007167:Immunotherapy / Q000379:methods*