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Journal article : Review

The role of plasma exchange in the treatment of refractory autoimmune neurological diseases: a narrative review

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.
Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1007/s11481-021-10004-9

Authors

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Role:
Author
ORCID:
0000-0003-0037-454X
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Institution:
University of Oxford
Division:
MSD
Department:
Clinical Neurosciences
Oxford college:
Corpus Christi College
Role:
Author
ORCID:
0000-0002-7667-9748


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Funder identifier:
https://ror.org/03x94j517
Grant:
MR/V007173/1


Publisher:
Springer
Journal:
Journal of Neuroimmune Pharmacology More from this journal
Volume:
16
Issue:
4
Pages:
806-817
Place of publication:
United States
Publication date:
2021-10-02
Acceptance date:
2021-07-22
DOI:
EISSN:
1557-1904
ISSN:
1557-1890
Pmid:
34599742


Language:
English
Keywords:
Subtype:
Review
Pubs id:
1203007
Local pid:
pubs:1203007
Deposit date:
2025-02-07
ARK identifier:

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