Autoantibodies in chronic inflammatory neuropathies: diagnostic and therapeutic implications

  title={Autoantibodies in chronic inflammatory neuropathies: diagnostic and therapeutic implications},
  author={Luis A Querol and J{\'e}r{\^o}me Devaux and Ricard Rojas-Garc{\'i}a and Isabel Illa},
  journal={Nature Reviews Neurology},
The chronic inflammatory neuropathies (CINs) are rare, very disabling autoimmune disorders that generally respond well to immune therapies such as intravenous immunoglobulin (IVIg). The most common forms of CIN are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with monoclonal gammopathy of unknown significance. The field of CIN has undergone a major advance with the identification of IgG4 autoantibodies directed… 

Autoantibodies in chronic inflammatory demyelinating polyradiculoneuropathy.

The discovery of autoantibodies against nodal and paranodal proteins has proven useful in clinical practice, has uncovered novel pathophysiological mechanisms, clinical phenotypes, therapeutic response and prognosis within the CIDP disease spectrum and has boosted the search for other clinically relevant autoantIBodies.

Diagnostic insights into chronic-inflammatory demyelinating polyneuropathies.

The present review provides a summary of the progress in autoantibody testing in CIDP and its possible implication on the stratification of the C IDP variants and treatment response.

Autoantibodies Against the Node of Ranvier in Seropositive Chronic Inflammatory Demyelinating Polyneuropathy: Diagnostic, Pathogenic, and Therapeutic Relevance

Current knowledge on the role of autoantibodies against proteins located at the node of Ranvier proteins and their clinical relevance in CIDP are reviewed with relevance for the emerging concept of nodopathy/paranodopathy.

Evolving Immunologic Perspectives in Chronic Inflammatory Demyelinating Polyneuropathy

Progress in search for antibodies specific to CIDP will enable earlier accurate diagnosis with direct management implications but only if the important, unfortunately and infrequently discussed issues of immunologic technique, test reliability and reproducibility are adequately tackled.

ARTHUR ASBURY LECTURE: Chronic inflammatory demyelinating polyradiculoneuropathy: clinical aspects and new animal models of auto‐immunity to nodal components

  • I. Illa
  • Biology, Medicine
    Journal of the peripheral nervous system : JPNS
  • 2017
Although paranodal and nodal autoantibodies are only found in a small subset of patients with CIDP, the detection of these immune biomarkers should be incorporated in the evaluation of patients, considering the implications of their presence on prognosis, follow-up, and treatment decisions.

Immune-mediated neuropathies

This Primer discusses the epidemiology, mechanisms, differential diagnosis and management of the immune-mediated neuropathies — a large group of disorders that includes Guillain–Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy, among other disorders.

Novel Immunological and Therapeutic Insights in Guillain-Barré Syndrome and CIDP

  • L. QuerolC. Lleixà
  • Medicine, Biology
    Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics
  • 2021
A topical review describes immunological progress that may help guide therapeutic strategies in the future in inflammatory neuropathies and Guillain-Barré syndrome, to optimize disease outcomes in CIDP and GBS.

Antecedent infections in CIDP: A relevant clue in a complex disease?

A possible relationship between preceding infections and CIDP in a large multinational cohort is reported and it is noted that infections may trigger dysimmune neuropathies that have more (sub)acute onset, but whether this is limited to specific infectious agents remains unclear and warrants confirmation in larger prospective cohorts.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Its Variants.

CIDP is characterized by progressive symmetric proximal and distal weakness, large fiber sensory loss, and areflexia, with clinical nadir reached more than 8 weeks after symptom onset, which allows for selection of the most appropriate treatment.

Distinguish CIDP with autoantibody from that without autoantibody: pathogenesis, histopathology, and clinical features

CIDP with autoantibodies should be considered as an independent disease entity, not a subtype of CIDP due to many differences, which can better characterize these disorders, help diagnose and make the most effective therapeutic decisions.



Paranodal and other autoantibodies in chronic inflammatory neuropathies.

The heterogeneity of chronic inflammatory neuropathies is being unraveled with the description of specific autoantibodies and their association with small disease subtypes, and the recently described paranodal autoantibia anti-CNTN1 and NF155 have direct clinical value and seem to determine response to treatment.

Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy

The data demonstrate that the pathogenic mechanisms responsible for chronic inflammatory demyelination polyneuropathy are broad and may include dysfunctions at the nodes of Ranvier in a subgroup of patients and that autoantibody depletion and recovery of conduction block and distal motor amplitude suggest a nodo-paranodopathy.

Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype

In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed and delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDs.

Autoimmune antigenic targets at the node of Ranvier in demyelinating disorders

The molecular anatomy of the node of Ranvier is explored, focusing on proteins with extracellular domains that could serve as antigens, and the clinical implications of node-specific antibody responses are addressed.

Diagnosis and treatment of chronic acquired demyelinating polyneuropathies

  • N. Latov
  • Medicine, Biology
    Nature Reviews Neurology
  • 2014
Chronic neuropathies are operationally classified as primarily demyelinating or axonal, on the basis of electrodiagnostic or pathological criteria, and hereditary or acquired—this distinction is important, because the acquired neuro Pathies are immune-mediated and, thus, amenable to treatment.

Chronic inflammatory demyelinating polyradiculoneuropathy and variants: where we are and where we should go

Several data support the role of the immune system in the pathogenesis of CIDP even if the precise targets and actors (antibodies and lymphocytes) of this immune response remain uncertain.

Pathogenesis and Treatment of Anti-MAG Neuropathy

  • M. Dalakas
  • Biology, Medicine
    Current treatment options in neurology
  • 2010
Open label studies and a recent randomized controlled trial indicate that rituximab is emerging as the best agent available, providing long-term benefits to almost half of these patients, and encouraging results need confirmation with a larger trial.

Neurofascin as a target for autoantibodies in peripheral neuropathies

Autoantibodies to NF are detected in a very small proportion of patients with AIDP and patients with CIDP, but may nevertheless be pathogenic in these cases.

Heterogeneity of Polyneuropathy Associated with Anti-MAG Antibodies

The data suggest that polyneuropathy associated with anti-MAG antibodies is less homogeneous than previously said and that the pathophysiology of the condition is likely to be heterogeneous as well with the self-antigen being MAG in most of the patients but possibly being another component of myelin in the others.