Current treatments of chronic immune‐mediated demyelinating polyneuropathies

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN), and anti-myelin-associated glycoprotein (anti-MAG) neuropathy are three demyelinating acquired neuropathies, with distinct responses to immunotherapy. In placebo-controlled, double-blind, randomized trials, intravenous immunoglobulin (IVIg) has been effective for CIDP and MMN, and plasmapheresis has been effective for CIDP. Corticosteroids have been beneficial in controlled trials for CIDP. Other agents, including cyclophosphamide, rituximab, azathioprine, cyclosporine, interferons, fludarabine, mycophenolate mofetil, and etanercept, have been reported to benefit some patients with inflammatory demyelinating neuropathies in case series and case reports. This review examines the use and toxicity associated with these immunotherapy medications in treating patients with chronic immune-mediated demyelinating neuropathies. Muscle Nerve, 2009.

Biological Agents for Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…Specific recommendations on management strategies are not proposed, as reviews on this subject have been published. [24][25][26][27][28] Biological therapeutics may rarely induce dysimmune inflammatory neuropathies (discussed elsewhere). …”

Auto-SCT in severe paraproteinemic neuropathy

“…Igs, plasmapheresis or immunosuppressive treatment (for example, corticosteroids, azathioprine or CY) and-rarely-immunoselective treatment using monoclonal antibodies (anti-CD20, anti-TNF-alpha and anti-CD52). 5 The response to treatment is variable, but most patients can be stabilized or even improved. 5 Nevertheless, a group of patients remain refractory to these treatment modalities, including some of those with anti-MAGassociated neuropathy.…”

“…5 The response to treatment is variable, but most patients can be stabilized or even improved. 5 Nevertheless, a group of patients remain refractory to these treatment modalities, including some of those with anti-MAGassociated neuropathy. 5 For these cases intensive immunosuppression followed by auto-SCT could be an alternative therapy concept.…”

Alemtuzumab: the future of chronic inflammatory demyelinating polyradiculoneuropathy treatment?

“…At some point during the course of the disease, 54% of patients are disabled [4]. Intravenous immune globulin (IVIg), plasmapheresis and prednisolone have all been studied in randomized placebo -controlled trials and are considered to be first-line treatments [5]. Not all patients respond to these treatments [6], and in some the response is suboptimal or there are significant side effects.…”

“…Not all patients respond to these treatments [6], and in some the response is suboptimal or there are significant side effects. In addition, the majority of patients require ongoing treatment; therefore, there is concern about the high cost of IVIg and plasma pheresis, the serious side effects associated with chronic corticosteroid use and the high cost of these side effects [5,[7][8][9].…”