Chronic Inflammatory Demyelinating Polyradiculoneuropathy - An Overview of Existing Treatment Options and Prospects for the Future

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated polyneuropathy. CIDP can cause prolonged periods of disability with many patients becoming severely debilitated at some time during their illness. Several open, uncontrolled studies and blinded, randomised clinical trials have shown that immunomodulatory therapy has a beneficial effect in CIDP. This paper reviews existing treatment options, provides a treatment algorithm and discusses prospects for the future. Corticosteroids, intravenous immunoglobulin, plasma exchange, immunosuppressive agents such as azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide, cyclosporine A, rituximab, alemtuzumab, etanercept, tacrolimus, interferon beta and alpha and autologous stem cell transplantation have been used in CIDP. Only corticosteroids, intravenous immunoglobulin and plasmapheresis have been proven to be effective in randomised controlled trials, however. As non-responsiveness to treatment seems to be associated with a greater degree of axonal dysfunction, finding therapies aimed at protecting the axon and restoring axonal damage are needed.

New Insights Into the Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…Some alternative approaches under investigation in this setting include corticosteroid-sparing and IVIG-sparing agents, stem cell transplantation and biological agents that target specific molecules involved in the immunopathology of CIDP [2,16,17]. Available evidence for azathioprine, interferon and methotrexate indicates little to no benefit with these agents in refractory CIDP [16,17].…”

“…Some alternative approaches under investigation in this setting include corticosteroid-sparing and IVIG-sparing agents, stem cell transplantation and biological agents that target specific molecules involved in the immunopathology of CIDP [2,16,17]. Available evidence for azathioprine, interferon and methotrexate indicates little to no benefit with these agents in refractory CIDP [16,17]. Considerable benefits with cyclophosphamide [18,19] and cyclosporine [20] were reported in small case series of patients with refractory CIDP, but the lack of new data in the past decade suggests that other research avenues are preferred.…”

“…Autologous hematopoietic stem cell transplantation induced long-term drug-free remission in 8 of 11 patients with therapy-refractory CIDP [21], but application of the procedure is limited by the risk of associated infections. Numerous biological agents directed against T cells, B cells and transmigration and transduction molecules (e.g., alemtuzumab, eculizumab, etanercept, fingolimod, natalizumab, rituximab and tacrolimus) have been investigated, with review Rajabally, Blomkwist-Markens & Katzberg future science group varying degrees of success [2,[16][17]. Of note, a retrospective, observational and multicenter study reported a favorable response to rituximab in 9 of 13 patients refractory to conventional therapies for CIDP [22].…”

Electrophysiological and neuromuscular stability of persons with chronic inflammatory demyelinating polyneuropathy

“…In inflammatory polyneuropathies such as CIDP, early diagnosis and therapy may not only mitigate progression of demyelination but also prevent secondary motor unit (MU) loss, which is often responsible for persisting motor symptoms. Therefore, a better understanding of the degree and time course of loss and stability of functioning MUs due either to primary or secondary demyelination in CIDP would provide more insight into the pathophysiology of the disease and possibly improve functional outcomes.…”