IVIg in other autoimmune neurological disorders: current status and future prospects.
A number of autoimmune disorders have been identified in which IVIg treatment may be beneficial. Evidence for the use of IVIg in inflammatory myopathies comes from controlled trials in dermatomyositis (DM) and sporadic-inclusion body myositis (s-IBM). In DM, muscle strength was increased and neuromuscular scores and skin rashes improved. Results for s-IBM have not been as encouraging as those observed for DM. Subsequently, IVIg should be recommended as a second-line therapy in DM and used for life-threatening dysphagia in s-IBM. Using an animal model of experimental autoimmune myasthenia gravis (MG), studies also indicate that IVIg can significantly improve clinical symptoms and affect pathogenic idiotypic antibodies. In human MG, studies indicate that IVIg exhibited equal efficacy compared to plasmapheresis. IVIg can therefore be recommended for use in an MG crisis or in lieu of plasmapheresis. The role of IVIg in the chronic management of MG has not been studied. IVIg has also been investigated in autoimmune CNS disorders. In a controlled study in patients with stiff person syndrome IVIg was effective, with improvements in the distribution of stiffness index and heightened sensitivity scores. For neurodegenerative diseases such as Alzheimer's disease, post-polio syndrome, pain, fibrosis, and autoimmune sleep disorders, some early promising results for the use of IVIg are emerging, but remain to be fully investigated. In conclusion, IVIg appears to be an effective treatment for a number of autoimmune disorders, however, optimal dosing and pharmacogenetic studies are necessary.
Dalakas
Marinos
M
Neuromuscular Division, Dept. of Neurology, 900 Walnut St., 2nd Floor, Philadelphia, PA 19107, USA. marinos.dalakas@jefferson.edu
D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000494:pharmacology*; D007167:Immunotherapy / Q000379:methods* / Q000639:trends; D023421:Models, Animal; D009135:Muscular Diseases / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D009157:Myasthenia Gravis / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D019636:Neurodegenerative Diseases / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D016750:Stiff-Person Syndrome / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous