Abstract
Autoimmune neurological disorders are commonly treated with immunosuppressive therapy. In patients with refractory conditions, standard immunosuppression is often insufficient for complete recovery or to prevent relapses. These patients rely on other treatments to manage their disease. While treatment of refractory cases differs between diseases, intravenous immunoglobulin, plasma exchange (PLEX), and immune-modulating treatments are commonly used. In this review, we focus on five autoimmune neurological disorders that were the themes of the 2018 Midlands Neurological Society meeting on PLEX in refractory neurology: Autoimmune Encephalitis (AE), Multiple Sclerosis (MS), Neuromyelitis Optica Spectrum disorders (NMOSD), Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and Myasthenia Gravis (MG). The diagnosis of inflammatory neuropathies is often challenging, and while PLEX can be very effective in refractory autoimmune diseases, its ineffectiveness can be confounded by misdiagnosis. One example is POEMS syndrome (characterized by Polyneuropathy Organomegaly, Endocrinopathy, Myeloma protein, Skin changes), which is often wrongly diagnosed as CIDP; and while CIDP responds well to PLEX, POEMS does not. Accurate diagnosis is therefore essential. Success rates can also differ within ‘one’ disease: e.g. response rates to PLEX are considerably higher in refractory relapsing remitting MS compared to primary or secondary progressive MS. When sufficient efforts are made to correctly pinpoint the diagnosis along with the type and subtype of refractory autoimmune disease, PLEX and other immunotherapies can play a valuable role in the patient management.Graphical abstract
Highlights
There are numerous autoimmune disorders involving the central nervous system (CNS) and the peripheral nervous system (PNS)
We focus on the five autoimmune neurological disorders that were presented at the meeting of 2018: Autoimmune Encephalitis (AE), Multiple Sclerosis (MS), Neuromyelitis Optica Spectrum Disorder (NMOSD), Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and Myasthenia Gravis (MG)
plasma exchange (PLEX) is recommended in the Academy of Neurology (AAN) (2011) guidelines for the treatment of fulminant CNS demyelinating diseases that fail to respond to high-dose corticosteroid treatment
Summary
There are numerous autoimmune disorders involving the central nervous system (CNS) and the peripheral nervous system (PNS). Patients with steroid-refractory relapses can benefit from PLEX, with reported response rates of 40 to 90% (Stork et al 2018). PLEX is recommended in the AAN (2011) guidelines for the treatment of fulminant CNS demyelinating diseases that fail to respond to high-dose corticosteroid treatment These CNS demyelinating diseases include MS, acute disseminated encephalomyelitis (ADEM), NMOSD and transverse myelitis; the study results did not allow to determine if effectiveness of PLEX varies between the different diseases (Cortese et al 2011). In a study on patients with chronic inflammatory neuropathies in southeast England, treatment response in patients with CIDP was observed for 68% of the patients treated with corticosteroids, for 63% of those treated with IVIg and for 42% of those treated with PLEX (Mahdi-Rogers and Hughes 2014). As an alternative for PLEX in long-term treatment, immunoadsorption can be very suitable, as its mechanism of action is more selective (Mantegazza and Antozzi 2018)
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