Rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy: A report of 13 cases and review of the literature

Department of Neurology, Osp. S. Andrea, Via Vittorio Veneto 197, 19100 La Spezia, Italy.
Journal of neurology, neurosurgery, and psychiatry (Impact Factor: 5.58). 03/2011; 82(3):306-8. DOI: 10.1136/jnnp.2009.188912
Source: PubMed

ABSTRACT A few case reports have shown controversial results of rituximab efficacy in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
To analyse the efficacy of rituximab in a large CIDP cohort.
A retrospective, observational and multicentre study on the use of rituximab in CIDP. 13 Italian CIDP patients were treated with rituximab after the partial or complete lack of efficacy of conventional therapies. Eight patients had co-occurring haematological diseases. Patients who improved by at least two points in standard clinical scales, or who reduced or discontinued the pre-rituximab therapies, were considered as responders.
Nine patients (seven with haematological diseases) responded to rituximab: six of them, who were non-responders to conventional therapies, improved clinically, and the other three maintained the improvement that they usually achieved with intravenous immunoglobulin or plasma exchange. Significantly associated with shorter disease duration, rituximab responses started after a median period of 2.0 months (range, 1-6) and lasted for a median period of 1 year (range, 1-5).
Rituximab seems to be a promising therapeutic choice when it targets both CIDP and co-occurring haematological diseases. Timely post-onset administration of rituximab seems to be associated with better responses.

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Available from: Cristoforo Comi, Aug 27, 2015
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    • "Investigators reported that six of 13 treatmentrefractory patients with CIDP responded to ritxuximab in an open-label, unblinded fashion [Benedetti et al. 2011]. Another retrospective open-label review from a nationwide CIDP patient registry suggested rituximab was beneficial in some patients [Cocito et al. 2011a]. "
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    ABSTRACT: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune mediated disorder of the peripheral nervous system with clinical features that include weakness, sensory loss, imbalance, pain and impaired ambulation which may lead to substantial disability. This review highlights current treatment strategies for CIDP, how best to utilize proven therapies such as intravenous immunoglobulin, oral prednisone, pulse dexamethasone, and plasma exchange, and when and how to use alternative immunosuppressive agents when first-line therapies are ineffective or poorly tolerated.
    Therapeutic Advances in Neurological Disorders 11/2012; 5(6):359-73. DOI:10.1177/1756285612457215
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    • "This family includes the monoclonal antibody directed against CD20, expressed on B cells (rituximab or the humanized version occrelizumab), and agents against B-cell growth factors, such as B-cell activating factor (BAFF) and a proliferating-inducing ligand (APRIL) (Dalakas, 2008a; 2008b). Rituximab, at a dose of 2 g given intravenously in two infusions, 15 days apart, or 375 mg/m 2 in 4 weekly infusions, is a promising agent; up to 50% of the treated CIDP patients seem to improve after 2–12 months (M ¨ unch et al., 2007; Briani et al., 2004; Kosmidis and Dalakas, 2010; Benedetti et al., 2011a; 2011b). Controlled studies are planned. "
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    ABSTRACT: The main chronic autoimmune neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and anti-myelin-associated glycoprotein (MAG) demyelinating neuropathy. On the basis of randomized controlled studies, corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis provide short-term benefits in CIDP. MMN responds only to IVIg. Because in MMN and CIDP, IVIg infusions are required every 3-6 weeks to sustain benefits or long-term remissions, there is a need for "IVIg-sparing" agents. In CIDP, immunosuppressive drugs, such as azathioprine, cyclosporine, methotrexate, mycophenolate, and cyclophosphamide, are used, but controlled trials have not shown that they are effective. Controlled trials have also not shown benefit to any agents in anti-MAG neuropathy. However, clinicians use many immunosuppressive drugs in both settings, but all have potentially serious side effects and are only effective in some patients. Thus, there is a need for new therapies in the inflammatory and paraproteinemic neuropathies. New agents targeting T cells, B cells, and transmigration and transduction molecules are discussed as potential treatment options for new trials. The need for biomarkers that predict therapeutic responses or identify patients with active disease is emphasized, and the search for better scoring tools that capture meaningful changes after response to therapies is highlighted.
    Journal of the Peripheral Nervous System 05/2012; 17 Suppl 2(2):34-9. DOI:10.1111/j.1529-8027.2012.00393.x · 2.50 Impact Factor
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    • "In our patients, motor conduction data did not improve despite improvement of the corresponding clinical scales (the INCAT overall disability sum score and MRC) as confirmed by other studies (Dalakas et al., 2009 Benedetti et al., 2011). Significant improvement in pin sensitivity but not in proprioceptive sensitivity was found after rituximab treatment. "
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    ABSTRACT: Rituximab treatment has shown clinical improvement in anti-myelin associated glycoprotein (MAG) polyneuropathy. We analyzed scores of clinical scales and the most sensitive electrophysiological parameters before and after immunomodulating treatment with rituximab in a group of patients affected by anti-MAG demyelinating polyneuropathy. Clinical scores, the percentage of CD20 B-lymphocytes, anti-MAG antibody titers and electrophysiological data in 7 patients with anti-MAG polyneuropathy were analyzed. The patients were examined before a cycle with rituximab, 6, 12 and 24 months after the end of the treatment. Two patients were treated with rituximab additional cycles and re-evaluated 48 months after the first treatment. There were no evident correlation between anti-MAG serum antibody titers or clinical scales and electrodiagnostic data. Significant decrease in the proportion of CD20 B-lymphocytes was observed. Significant anti-MAG antibodies titers reduction was detected after re-treatment. At follow-up, pinprik sensation and two point discrimination presented a significant improvement compared with the score before treatment. In our patients, rituximab did not improve any electrophysiological data. No correlation with anti-MAG serum antibodies course was found. With rituximab only pin sensibility improved. Rituximab re-treatment significantly reduces anti-MAG serum antibodies titers but improves only small fibers sensibility.
    Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 06/2011; 122(12):2518-22. DOI:10.1016/j.clinph.2011.05.015 · 2.98 Impact Factor
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