Electrophysiological features of chronic inflammatory demyelinating polyradiculoneuropathy associated with IgG4 antibodies targeting neurofascin 155 or contactin 1 glycoproteins

Kouton, Ludivine; Boucraut, José; Devaux, Jérôme; Rajabally, Yusuf A.; Adams, David H.; Antoine, J. C.; Bourdain, Frédéric; Brodovitch, Alexandre; Camdessanché, Jean‐Philippe; Cauquil, Cécile; Ciron, Jonathan; Dubard, Thierry; Echaniz‐Laguna, Andoni; Grapperon, Aude-Marie; Juntas-Moralès, Raul; Kremer, Laurent; Küntzer, Thierry; Labeyrie, Céline; Lanfranco, Luca; Léger, Jean‐Marc; Maisonobe, Thierry; Mavroudakis, Nicolas; Mecharles-Darrigol, Sylvie; Merle, P; Noury, Jean-Baptiste; Rouaud, Violaine; Tard, Céline; Théaudin, Marie; Vallat, Jean‐Michel; Viala, Karine; Attarian, Shahram; Delmont, Émilien

doi:10.1016/j.clinph.2020.01.013

Cited by 45 publications

(30 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Antibodies against Nfasc155, CNTN1, Nfasc186 and Casp1 were detected with a flow cytometry technique using human embryonic kidney (HEK) cells transfected with the respective plasmid of interest [3,13,21]. All the sera were first screened for IgG4 isotype antibodies against the node of Ranvier.…”

Section: Methodsmentioning

confidence: 99%

Antibodies against the node of Ranvier: a real-life evaluation of incidence, clinical features and response to treatment based on a prospective analysis of 1500 sera

et al. 2020

Self Cite

View full text Add to dashboard Cite

Introduction IgG4 antibodies against neurofascin (Nfasc155 and Nfasc140/186), contactin (CNTN1) and contactin-associated protein (Caspr1) are described in specific subtypes of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Our objective was to assess, in a real-life practice, the incidence, the clinical features and the response to treatment of these forms of CIDP. Methods 1500 sera of patients suspected of having CIDP from France, Belgium and Switzerland were prospectively tested using a flow cytometry technique. The characteristics of patients with antibodies against the node of Ranvier were compared to 100 seronegative CIDP from our department. Results IgG4 antibodies against Nfasc155, CNTN1, and Caspr1 were, respectively, detected in 15 (prevalence 1%), 10 (0.7%) and 2 (0.2%) sera. Antibodies specific of the Nfasc140/186 were not detected. All subjects with antibodies against the node of Ranvier fulfilled diagnostic criteria for CIDP. CIDP with anti-Nfasc155 were younger, had more sensory ataxia and postural tremor than seronegative CIDP. CIDP with anti-CNTN1 had more frequent subacute onset and facial paralysis, commoner renal involvement with membranous glomerulonephritis and greater disability, than seronegative CIDP. CIDP with anti-Caspr1 had more frequent respiratory failure and cranial nerve involvement but not more neuropathic pain than seronegative CIDP. Intravenous immunoglobulins were ineffective in most seropositive patients. Rituximab produced dramatic improvement in disability and decreased antibodies titres in 13 seropositive patients (8 with anti-Nfasc155 and 5 with anti-CNTN1 antibodies). Conclusions Although rare, anti-paranodal antibodies are clinically valuable, because they are associated with specific phenotypes and therapeutic response.

show abstract

Section: Methodsmentioning

confidence: 99%

Antibodies against the node of Ranvier: a real-life evaluation of incidence, clinical features and response to treatment based on a prospective analysis of 1500 sera

et al. 2020

Self Cite

View full text Add to dashboard Cite

show abstract

“…NF155 is important for attaching the myelin sheath lamellae to the axon at the paranodes adjacent to the nodes of Ranvier. This leads to disruption of action potentials (conduction slowing and block) and consecutively to chronic neuropathies [chronic inflammatory demyelinating polyneuropathy; CIDP (63)(64)(65)(66)(67)]. In IgG4-AID, which target synaptic antigens localized in the peripheral and central nervous system, e.g.…”

Section: Known Igg4 Autoantibody-associated Disease: a Colorful Bouquetmentioning

confidence: 99%

Common Denominators in the Immunobiology of IgG4 Autoimmune Diseases: What Do Glomerulonephritis, Pemphigus Vulgaris, Myasthenia Gravis, Thrombotic Thrombocytopenic Purpura and Autoimmune Encephalitis Have in Common?

et al. 2021

View full text Add to dashboard Cite

IgG4 autoimmune diseases (IgG4-AID) are an emerging group of autoimmune diseases that are caused by pathogenic autoantibodies of the IgG4 subclass. It has only recently been appreciated, that members of this group share relevant immunobiological and therapeutic aspects even though different antigens, tissues and organs are affected: glomerulonephritis (kidney), pemphigus vulgaris (skin), thrombotic thrombocytopenic purpura (hematologic system) muscle-specific kinase (MuSK) in myasthenia gravis (peripheral nervous system) and autoimmune encephalitis (central nervous system) to give some examples. In all these diseases, patients’ IgG4 subclass autoantibodies block protein-protein interactions instead of causing complement mediated tissue injury, patients respond favorably to rituximab and share a genetic predisposition: at least five HLA class II genes have been reported in individual studies to be associated with several different IgG4-AID. This suggests a role for the HLA class II region and specifically the DRβ1 chain for aberrant priming of autoreactive T-cells toward a chronic immune response skewed toward the production of IgG4 subclass autoantibodies. The aim of this review is to provide an update on findings arguing for a common pathogenic mechanism in IgG4-AID in general and to provide hypotheses about the role of distinct HLA haplotypes, T-cells and cytokines in IgG4-AID.

show abstract

“…What is the association between these autoantibodies and the prognosis of CIDP patients? There are a total of 20 studies listed in Table 5 (Ng et al, 2012;Kawamura et al, 2013;Querol et al, 2013Querol et al, , 2014Yan et al, 2014;Doppler et al, 2015Doppler et al, , 2016Miura et al, 2015;Ogata et al, 2015Ogata et al, , 2019Devaux et al, 2016;Kadoya et al, 2016;Delmont et al, 2017;Mathey et al, 2017;Burnor et al, 2018;Zhang et al, 2019;Cortese et al, 2020;Godil et al, 2020;Kouton et al, 2020;Muley et al, 2020). Among these 20 studies, 5 studies were conducted in multiple countries, and the remaining 15 studies were single-country studies.…”

Section: Study Characteristicsmentioning

confidence: 99%

A Systematic Review and Meta-Analysis of Autoantibodies for Diagnosis and Prognosis in Patients With Chronic Inflammatory Demyelinating Polyradiculoneuropathy

et al. 2021

View full text Add to dashboard Cite

Objectives: To review the available evidence on sensitivity and specificity of anti-NF155 antibody detection in diagnosing a specific subset of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and to calculate the frequencies of different autoantibodies to paranodal proteins.Background: Diagnosis of CIDP relies on clinical and neurophysiologic criteria and lacks useful diagnostic biomarkers. A subset of CIDP patients exhibit atypical clinical phenotypes and impaired response to conventional treatments. These patients were reported as having autoantibodies targeting paranodal protein neurofascin isoform 155 (NF155), contactin-1 (CNTN1), and contactin-associated protein-1 (CASPR1). Here, we conducted a meta-analysis to summarize evidence on the diagnostic and prognostic value of these autoantibodies, especially for anti-NF155 antibody.Methods: We searched the following electronic bibliographic databases: PubMed, EMBASE, Cochrane Central Register of Controlled Trials (CENTRAL), and Web of Science. Eligible studies provided information to calculate the frequencies of anti-NF155 antibody and anti-CNTN1 antibody, the sensitivity and specificity of anti-NF155 antibody, and the incidence of improvement and deterioration among anti-NF155 antibody seropositive CIDP patients. Heterogeneity was assessed using Q and I2 statistics.Results: The pooled frequency of anti-NF155 autoantibody across 14 studies was 7% [95% confidence interval (CI): 0.05–0.10] with high heterogeneity; the overall pooled sensitivity and specificity of anti-NF155 antibody for the diagnosis of a specific subgroup of CIDP patients were 0.45 (95% CI: 0.29–0.63) and 0.93 (95% CI: 0.86–0.97), respectively.Conclusions: For diagnosing of a specific subset of CIDP characterized by poor response to intravenous immunoglobulin (IVIg), we found a moderate sensitivity and a high specificity. The anti-NF155 antibody test should be used as a confirmatory test rather than a screening test.Systematic Review Registration: PROSPERO, identifier: CRD42020203385 and CRD42020190789.

show abstract

Electrophysiological features of chronic inflammatory demyelinating polyradiculoneuropathy associated with IgG4 antibodies targeting neurofascin 155 or contactin 1 glycoproteins

Cited by 45 publications

References 26 publications

Antibodies against the node of Ranvier: a real-life evaluation of incidence, clinical features and response to treatment based on a prospective analysis of 1500 sera

Antibodies against the node of Ranvier: a real-life evaluation of incidence, clinical features and response to treatment based on a prospective analysis of 1500 sera

Common Denominators in the Immunobiology of IgG4 Autoimmune Diseases: What Do Glomerulonephritis, Pemphigus Vulgaris, Myasthenia Gravis, Thrombotic Thrombocytopenic Purpura and Autoimmune Encephalitis Have in Common?

A Systematic Review and Meta-Analysis of Autoantibodies for Diagnosis and Prognosis in Patients With Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Contact Info

Product

Resources

About