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: 6.81). 03/2011; 82(3):306-8. DOI: 10.1136/jnnp.2009.188912
Source: PubMed


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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    • "According to uncontrolled studies [123–125] Rituximab might be helpful in CIDP associated with hematological disorders such as monoclonal gammopathy of undetermined significance and multiple myeloma, but a very recent RCT [126] on 54 patients with anti-MAG followed up for one year has shown no significant benefit from Rituximab compared to placebo in terms of changes in the ISS score [78]. Rituximab also failed as an IVIg-sparing agent in patients dependent on IVIg [127]. "
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    ABSTRACT: 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.
    Full-text · Article · Jan 2014
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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.
    Full-text · Article · Nov 2012 · Therapeutic Advances in Neurological Disorders
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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.
    Preview · Article · May 2012 · Journal of the Peripheral Nervous System
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