Advances in the diagnosis, pathogenesis and treatment of CIDP

Neuroimmunology Unit, Department of Pathophysiology, National University of Athens Medical School, Building 16, Room 39, 75 Mikras Asias Street, Athens 11527, Greece.
Nature Reviews Neurology (Impact Factor: 15.36). 08/2011; 7(9):507-17. DOI: 10.1038/nrneurol.2011.121
Source: PubMed


Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. Despite clinical challenges in diagnosis-owing in part to the existence of disease variants, and different views on how many electrophysiological abnormalities are needed to document demyelination-consensus criteria seem to have been reached for research or clinical practice. Current standard of care involves corticosteroids, intravenous immunoglobulin (IVIg) and/or plasmapheresis, which provide short-term benefits. Maintenance therapy with IVIg can induce sustained remission, increase quality of life and prevent further axonal loss, but caution is needed to avoid overtreatment. Commonly used immunosuppressive drugs offer minimal benefit, necessitating the development of new therapies for treatment-refractory patients. Advances in our understanding of the underlying immunopathology in CIDP have identified new targets for future therapeutic efforts, including T cells, B cells, and transmigration and transduction molecules. New biomarkers and scoring systems represent emerging tools with the potential to predict therapeutic responses and identify patients with active disease for enrollment into clinical trials. This Review highlights the recent advances in diagnosing CIDP, provides an update on the immunopathology including new target antigens, and discusses current treatments, ongoing challenges and future therapeutic directions.

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    • "In conclusion, the chronic-EAN model we have developed is a reliable and reproducible model that may prove useful for the study of new immunotherapeutic strategies and for translational drug studies for chronic human autoimmune-mediated inflammatory diseases of the PNS, and particularly CIDP, for which, there is a need of new targeted immunotherapies (Dalakas, 2011). "
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    ABSTRACT: Animal model Thiopalmitoylated P0 peptide Chronic EAN CIDP Electrophysiology Sciatic nerve immunohistochemistry Immunology Our objective was to develop a chronic model of EAN which could be used as a tool to test treatment strategies for CIDP. Lewis rats injected with S-palmitoylated P0(180–199) peptide developed a chronic, sometimes relapsing– remitting type of disease. Our model fulfills electrophysiological criteria of demyelination with axonal degener-ation, confirmed by immunohistopathology. The late phase of the chronic disease was characterized by accumu-lation of IL-17 + cells and macrophages in sciatic nerves and by high serum IL-17 levels. In conclusion, we have developed a reliable and reproducible animal model resembling CIDP that can now be used for translational drug studies.
    Journal of Neuroimmunology 01/2015; 278:1-10. DOI:10.1016/j.jneuroim.2014.11.022 · 2.47 Impact Factor
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    • "Various modalities of treatment such as immunomodulating drugs, intravenous immunoglobulin (IVIG), steroids and plasmapheresis have been used for the treatment of this disease [2]. Additionally, cyclophosphamide is used in patients who did not respond to IVIG, steroids or plasmapheresis [3]. "
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    ABSTRACT: Although various modalities of treatment of chronic inflammatory demyelinating polyradiculopathy (CIDP) there are not any treatment protocol agreed. We retrospectively evaluated the 20 CIDP patients (14 male, 6 female). Five patients were excluded from the study because they could not continue their treatments due to various problems during the treatment. The remaining 15 patients treated with monthly high dose intravenous methyl prednisolone for five years (IVMP) and followed up for 10 years. The mean age of the patients was 48.1±14.6 years. The mean duration of disease was 6.8±3.1 years. We were found statistically significant difference between the pre-treatment and sixth month modified Rankin scores (p<0.001). Similarly, significant improvement was observed at the end of first, fourth and fifth years of treatment. Statistically significant difference was found between baseline and tenth year modified Rankin scores of 12 patients who were treated with only IVMP during 10 years follow-up. Long-term monthly IVMP pulse therapy seems to be very effective in the treatment of CIDP.
    Clinical neurology and neurosurgery 03/2014; 118C:89-93. DOI:10.1016/j.clineuro.2014.01.001 · 1.13 Impact Factor
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    • "It is the most common peripheral autoimmune demyelinating neuropathy with a prevalence of 1.2 to 7.7 per 100,000 worldwide, with a slight male predominance [2]. The disease involves progressive loss of immunologic tolerance to peripheral nerve components such as myelin, Schwann cell, the axon, and motor or ganglionic neurons [3,4]. There is increasing evidence that activated macrophages, T cells, and auto-antibodies induce an immune attack against peripheral nerve antigens [4]. "
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    ABSTRACT: Chronic inflammatory demyelinating polyradiculoneuropathy is a rare acquired immune-mediated progressive or relapsing disorder causing peripheral neuropathic disease of duration more than two months. Many individuals with chronic inflammatory demyelinating polyradiculoneuropathy fail to make a long-term recovery with current treatment regimes. The aim of this study was to prospectively review the literature to determine the effectiveness of therapies for chronic inflammatory demyelinating polyradiculoneuropathy. Articles published from January 1990 to December 2012 were searched for studies to treat adults with chronic inflammatory demyelinating polyradiculoneuropathy. Peer-reviewed full-text articles published in English were included. Nine placebo-controlled double-blinded randomised trials were reviewed to treat subjects with chronic inflammatory demyelinating polyradiculoneuropathy exhibiting various degrees of effectiveness. The most effect treatments were; three randomised controlled trials using intravenous immunoglobulin, a study comparing pulsed dexamethasone and short term prednisolone and rituximab all showed promising results and were well tolerated. IVIg and corticosteroids remain first line treatments for CIDP. Therapies using monoclonal antibodies, such as Rituximab and Natalizumab offer the most promise for treatment of Chronic inflammatory demyelinating polyradiculoneuropathy however they also need further research, as does the use of stem cell therapy for treating Chronic inflammatory demyelinating polyradiculoneuropathy. Large randomised controlled trials and better patient selection are required to address responsiveness of CIDP patients to conventional treatments to elucidate mechanisms of action and future directions for therapeutic improvement.
    BMC Neurology 02/2014; 14(1):26. DOI:10.1186/1471-2377-14-26 · 2.04 Impact Factor
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