General principles and escalation options of immunotherapy in autoantibody-associated disorders of the CNS
- PDF / 1,035,189 Bytes
- 10 Pages / 595.276 x 790.866 pts Page_size
- 50 Downloads / 162 Views
(2019) 1:32
Neurological Research and Practice
REVIEW
Open Access
General principles and escalation options of immunotherapy in autoantibodyassociated disorders of the CNS Ilya Ayzenberg1,2*, Simon Faissner1, Laura Tomaske1, Daniel Richter1, Volker Behrendt1 and Ralf Gold1
Abstract Autoimmune diseases associated with antineuronal and antiglial autoantibodies (Abs) is one of the most rapidly expanding research fields in clinical neuroimmunology, with more than 30 autoantibodies described so far. Being associated with a wide range of clinical presentations these syndromes can be diagnostically challenging. Surface or intracellular antigen localizations are crucial for the treatment response and outcome. In the latter Abs are mostly of paraneoplastic cause and tumor management should be performed as soon as possible in order to stop peripheral antigen stimulation. Immunotherapy should be started early in both groups, before irreversible neuronal loss occurs. Despite serious prognosis, aggressive therapeutic approaches can be effective in many cases. In this article we review main pathogenic mechanisms leading to Abs-related syndromes and describe standard as well as emerging strategies of immunotherapy, including tocilizumab and bortezomib. Several special therapeutic approaches will be illustrated by clinical cases recently treated in our department.
Introduction Autoimmune diseases associated with antineuronal and antiglial autoantibodies (Abs) is one of the most rapidly expanding research fields in clinical neuroimmunology. Autoantibodies targeting more than 30 specific antigens in the central nervous systems (CNS) have been identified so far and several new candidate antigens are reported every year [1]. Intracellular or surface target protein localisation and in the last case its function often determine clinical presentation as well as key immunological mechanisms and accordingly preferable therapeutic approaches. Rarity and clinical diversity of the Abs-associated syndromes, lack of specific clinical features and partly overlapping symptoms are challenging for diagnostics. Studies on management are generally limited and large double-blind clinical trials have been conducted in neuromyelitis optica spectrum disorder (NMOSD) only [2]. Experience gathered from NMOSD management is useful, however, cannot be automatically transferred to other diseases associated * Correspondence: [email protected] 1 Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany 2 Department of Neurology, Sechenov First Moscow State Medical University, Moscow, Russia
with antineuronal Abs due to pathogenic differences. Treatment recommendations are mostly based on retrospective case series and expert opinions. Although several generally accepted therapy principles have been elaborated in the last decades, an individual strategy is often required, especially in rare syndromes and/or therapy refractory cases. Here, we discuss principles of management of Abs-associated CNS-diseases and describe several c
Data Loading...
