title: Therapeutic complement inhibition: a promising approach for treatment of neuroimmunological diseases.
abstract: Autoimmunity is an important cause of disease both in the central and peripheral nervous systems. Aetiologies and clinical manifestations are complex and heterogeneous. Inappropriate control of complement activation at inappropriate sites has been recognized as a major determinant in several neurological conditions, including Guillain-Barré syndrome and neuromyelitis optica. In each case pathogenesis is thought to be associated with generation of autoantibodies which upon binding guide activation of the complement system to self-tissue. Areas covered: Modulation of the complement system activation at such sites may represent a novel therapeutic approach for treatment of immune-mediated inflammatory conditions. In this review we focus on the therapeutic effects of complement inhibitors in Guillain-Barré syndrome and neuromyelitis optica and highlight recent developments within the field. Expert Commentary: Conventional first line treatment strategies in GBS and NMO have the potential disadvantage of causing widespread immunosuppressive effects. A more targeted approach may therefore be more effective and less disruptive to the immune system, especially in the case of NMO, which requires long term immunosuppression. Modulation of the complement system may hold the key and has already been shown to be of clinical benefit in other non-neurological conditions, including paroxysmal nocturnal hemoglobinuria and hereditary angioedema.
authors.affiliation: a Department of Cell and Developmental Biology , University College London , UK.
authors.forename: Kjara S
authors.initials: KS
authors.lastname: Pilch
authors.affiliation: b University of Bern, Institute of Pharmacology , Bern , Switzerland.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Spaeth
authors.affiliation: c Department of Neurology , University of Mishima , Niigata , Japan.
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Yuki
authors.affiliation: d Department of Neurology , Gloucestershire Hospitals NHS Foundation Trust , Gloucester , United Kingdom of Great Britain and Northern Ireland.
authors.forename: Benjamin R
authors.initials: BR
authors.lastname: Wakerley
mesh_terms: D001323:Autoantibodies; D015551:Autoimmunity; D003167:Complement Activation / Q000187:drug effects*; D020275:Guillain-Barre Syndrome / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D056747:Immunomodulation; D009471:Neuromyelitis Optica / Q000276:immunology* / Q000628:therapy*
chemical_list: D001323:Autoantibodies
keywords: Complement; Guillain-Barré syndrome; Miller Fisher Syndrome; eculizumab; neuromyelitis optica