Inflammatory polyradiculoneuropathies: Clinical and immunological aspects, current therapies, and future perspectives (original) (raw)
Related papers
Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype
Journal of Neurology, Neurosurgery, and Psychiatry, 2015
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves, the relative contributions of T cell and autoantibody responses remain largely undefined. In animal models of spontaneous inflammatory neuropathy, T cell responses to defined myelin antigens are responsible. In other human inflammatory neuropathies, there is evidence of antibody responses to Schwann cell, compact myelin or nodal antigens. In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed. In time, it is anticipated that delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDP.
A review on Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Zenodo (CERN European Organization for Nuclear Research), 2023
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a kind of inflammatory neuropathy that has a gradual start and symmetrical sensory involvement. However, there are several clinical variances, suggesting that CIDP may represent a spectrum of linked disorders rather than a single disease entity. While the prevailing idea of CIDP pathogenesis is that cell-mediated and humoral processes interact in an abnormal immune response to damage peripheral neurons, the proportional roles of T cell and autoantibody responses are yet unknown. T cell responses to specified myelin antigens are responsible in animal models of spontaneous inflammatory neuropathy. Antibodies to Schwann cell, compact myelin, and nodal antigens have been found in different human inflammatory neuropathies. The roles of the cellular and humoral immune systems in the development of CIDP are discussed in this review. It is believed that, in the future, the identification of clinical phenotypes and the underlying disease processes would aid in the development of diagnostic and therapy options for CIDP.
The Italian Journal of Neurological Sciences, 1991
An outline of the principal reports dealing with the definition, distribution, course and treatment of the inflammatory polyradiculoneuropathies, including the Guillain-Barr~ syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP), is given. Current diagnostic criteria for GBS are reaffirmed while the diagnosis of CIDP lacks proper standardization. Then, the bounderies between the two disorders are ill-defined. While GBS is rare and homogeneously distributed across developed and developing countries, the prevalence rate of CIDP is unknown. Several antecedent events have been implicated in the pathogenesis of GBS; yet, except for the swine-flu vaccine, the relation between infectious or toxic agents and the occurrence of the disease is purely anecdotal. The only factors known to influence the outcome of GBS are age, severity of opening symptoms, abnormal electrophysiologic characteristics of peripheral nerve func
Journal of Neuroimmunology, 2005
The role of antigen-presenting cells (APC) involved in induction of T and B cell mediated autoaggressive immunity in Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is poorly understood. We studied the numbers and phenotype of dendritic cells (DC) in blood and cerebrospinal fluid (CSF) over the course of GBS and CIDP before and after immunomodulatory treatment. Four out of seven GBS patients examined prior to treatment with high-dose intravenous immunoglobulins (IvIg) had elevated numbers of CD123 + plasmacytoid DC in the CSF, while both GBS and CIDP patients examined prior to treatment had elevated numbers of CD11c + myeloid DC in the CSF, as compared to patients with noninflammatory neurological diseases (OND). The percentages of blood DC expressing the cell surface marker CD1a, co-stimulatory molecules CD80 and CD86, adhesion molecule CD54, and chemokine receptors CCR1, CCR2, CCR5, and CXCR4 were not affected in GBS or CIDP. The immunohistochemistry of sural nerve biopsies revealed CD11c + CD83 À CD14 À CD16 À immature myeloid DC at low numbers, mostly in the perineurium, without difference between CIDP patients and controls. In contrast, the numbers of CD11c + CD14 + /CD16 + macrophages were higher within the endoneurium in CIDP patients compared with the controls. The recruitment of DC to CSF in GBS and CIDP may be important in capturing antigens released from inflamed spinal nerve roots into CSF and in transferring these antigens from CSF to local lymph nodes, where naRve T and B cells may be activated.
Journal of the Peripheral Nervous System, 2010
We retrospectively analyzed 146 patients fulfilling the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to (1) evaluate the relevance of these criteria, (2) assess the frequency of CIDP variants, and (3) determine the response to treatment and the prognosis. We found that 75% of these patients fulfilled the main EFNS/PNS clinical and electrophysiological criteria (type I). The remaining patients were diagnosed using laboratory tools as supportive criteria. The common form of CIDP represented 51% of patients. We observed a high frequency of the sensory variant (35% of patients) and the rapid onset form (18%). A positive response to treatment was observed in 87% of patients, with a similar efficacy of prednisone and IVIg. However, in the long term, 40% of treated patients remained dependent on treatment. The IVIg dependency rate was higher than the prednisone or plasma exchange dependency rate (55%, 18%, and 23%, respectively; p = 0.0054). Severe handicap was observed in 24% of patients.
Case Reports in Neurology
Acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is an immune mediated neuropathy characterized by progressive weakness and sensory impairment lasting over 2 months. Guillain-Barré-Strohl syndrome (GBS) is an immune mediated polyneuropathy with a similar presentation often over less than 4 weeks. While some have argued for the existence of recurrent GBS, most classify the syndrome as a form of relapsing-remitting CIDP. However, there are cases of GBS with treatment-related fluctuations that must be distinguished from A-CIDP as patients with A-CIDP require long-term immunotherapy. In this case report, we discuss a patient with multiple relapses over 3 years, who is more likely to have A-CIDP. His ganglioside profile, which has rarely been reported in A-CIDP, included high concentrations of anti-GM1, anti-GD1a, and anti-GD1b antibodies, which may account for his severe disease course.
International journal of scientific research, 2019
Inflammatory neuropathies such as Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are well-known immune-mediated polyneuropathies sharing many common characteristics during the acute disease phase. Autoimmune disorders are believed to develop as a result of interplay between genetic and environmental factors. GBS is often associated with preceding infections such as Campylobacter jejuni (C. jejuni), Mycoplasma pneumoniae, Epstein-Barr virus, cytomegalovirus (CMV), and varicella zoster virus (VZV). 1 In contrast, it is challenging to find an antecedent infection as a specific triggering event for CIDP, even in acute-onset CIDP (A-CIDP). 2 This might be due to environmental factors influencing the development of GBS more than the development of CIDP, and the immune system of CIDP patients being genetically permissive to be activated and, once activated, letting autoreactive T-cells remain viable and activated to cause a chronic autoimmune disease. 3,4 Here we describe a patient who had initially been diagnosed with GBS following hepatitis A virus (HAV) infection, but whose diagnosis was eventually changed to A-CIDP.
2014
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, in which both cellular and humoral immune responses are involved. The disease is clinically heterogeneous with some patients displaying pure motor form and others also showing a variable degree of sensory dysfunction; disease evolution may also differ from patient to patient, since monophasic, progressive, and relapsing forms are reported. Underlying such clinical variability there is probably a broad spectrum of molecular dysfunctions that are and will be the target of therapeutic strategies. In this review we first explore the biological bases of current treatments and subsequently we focus on the practical management that must also take into account pharmacoeconomic issues.