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Autoimmune Peripheral Neuropathies and Contribution of Antiganglioside/Sulphatide Autoantibody Testing.

ABSTRACT: Peripheral immune-mediated polyneuropathies (IMPN) are a diverse group of rare neurological illnesses characterized by nerve damage. Leading morphological features are mostly nerve fibre demyelination or combination of axonal damage and demyelination. There has been remarkable progress in the clinical and electrophysiological categorization of acute (fulminant, life-threatening) and chronic (progressive/remitting-relapsing) immune-mediated neuropathies recently. Besides electrophysiological and morphological makers, autoantibodies against glycolipids or paranodal/nodal molecules have been recommended as candidate markers for IMPN. The progress in testing for autoantibodies (autoAbs) to glycolipids such as gangliosides and sulfatide may have significant implications on the stratification of patients and their treatment response. Thus, this topic was reviewed in a presentation held during the 1st Panhellenic Congress of Autoimmune Diseases, Rheumatology and Clinical Immunology in Portaria, Pelion, Greece. For acute IMPN, often referred to as Guillain-Barré syndrome and its variants, several serological markers including autoAbs to gangliosides and sulphatide have been employed successfully in clinical routine. However, the evolution of serological diagnosis of chronic variants, such as chronic inflammatory demyelinating polyneuropathy or multifocal motor neuropathy, is less satisfactory. Serological diagnostic markers could, therefore, help in the differential diagnosis due to their assumed pathogenic role. Additionally, stratification of patients to improve their response to treatment may be possible. In general, a majority of patients respond well to causal therapy that includes intravenous immunoglobulins and plasmapheresis. As second line therapy options, biologicals (e.g., rituximab) and immunosuppressant or immunomodulatory drugs may be considered when patients do not respond adequately.

SUBMITTER: Roggenbuck D

PROVIDER: S-EPMC7219652 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature

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Autoimmune Peripheral Neuropathies and Contribution of Antiganglioside/Sulphatide Autoantibody Testing.

Roggenbuck Dirk D Delmont Emilien E Reinhold Dirk D Schierack Peter P Conrad Karsten K Boucraut Joseph J

Mediterranean journal of rheumatology 20200331 1

Peripheral immune-mediated polyneuropathies (IMPN) are a diverse group of rare neurological illnesses characterized by nerve damage. Leading morphological features are mostly nerve fibre demyelination or combination of axonal damage and demyelination. There has been remarkable progress in the clinical and electrophysiological categorization of acute (fulminant, life-threatening) and chronic (progressive/remitting-relapsing) immune-mediated neuropathies recently. Besides electrophysiological and ...[more]

PMID: 32411930

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Project description:Autoimmune diseases of the peripheral nervous system have so far been treated mainly with exogenous high-dose intravenous immunoglobulins (IVIg), that act through several mechanisms, including neutralization of pathogenic autoantibodies, modulation of lymphocyte activity, interference with antigen presentation, and interaction with Fc receptors, cytokines, and the complement system. Other therapeutic strategies have recently been developed, in part to address the increasing shortage of IVIg, prime among which is the use of B cell depleting monoclonal antibodies, or small molecule inhibitors targeting the B-cell specific kinases. Rituximab, a chimeric monoclonal antibody against CD20 + B lymphocytes, is currently the most used, especially in anti-MAG antibody neuropathy and autoimmune neuropathies with antibodies to nodal/paranodal antigens that are unresponsive to IVIg. After several reports of its efficacy in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), rituximab is currently under investigation in three Phase 2 trials in CIDP. In addition, the possible role of complement activation in the pathogenesis of chronic autoimmune neuropathies has brought into consideration drugs that can block the complement cascade, such as eculizumab, a monoclonal antibody already assessed in acute polyradiculoneuropathies, and approved for myasthenia gravis. Preliminary data on eculizumab in multifocal motor neuropathy have been published, but randomized controlled studies are pending. Moreover, the neonatal Fc receptor, that recycles IgGs by preventing their lysosome degradation, is an important and attractive pharmacological target. Antibodies against FcRn, which reduce circulating IgG (both pathogenic and non-pathogenic) have been developed. The FcRn blocker efgartigimod, a humanized IgG1-derived Fc fragment, which competitively inhibits the FcRn, has recently been approved for the treatment of myasthenia gravis and is currently under investigation in CIDP. In addition, the anti-human FcRn monoclonal antibody rozanolixizumab is currently being assessed in phase 2 trials in CIDP. However, none of the abovementioned monoclonal antibodies is currently approved for treatment of any immune-mediated neuropathies. While more specific and individualized therapies are being developed, the possibility of combined treatments targeting different pathogenic mechanisms deserves consideration as well.

Dataset Information

Autoimmune Peripheral Neuropathies and Contribution of Antiganglioside/Sulphatide Autoantibody Testing.

Publications

Autoimmune Peripheral Neuropathies and Contribution of Antiganglioside/Sulphatide Autoantibody Testing.

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