Chronic inflammatory demyelinating polyneuropathies: Current treatment strategies

Abstract

Chronic inflammatory demyelinating polyradiculopathy (CIDP), considered an immune-mediated disease, is likely under-recognized and under-treated due to its heterogeneous presentation and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria. Despite these limitations, early diagnosis and treatment is important in preventing irreversible axonal loss and improving functional recovery. Primary treatment modalities include intravenous immunoglobulin and plasmapheresis, for which there is randomized, double-blind, placebo-controlled evidence. In addition, despite less definitive published evidence of efficacy, corticosteroids are considered standard therapies because of their long history of use. Studies have failed to demonstrate a difference in efficacy among these three treatments; consequently, the choice is usually based on availability and side-effect profile. A number of chemotherapeutic and immunosuppressive agents have also shown to be effective in treating CIDP but significant evidence is lacking; therefore, these agents are primarily used in conjunction with other modalities. Regardless of the treatment choice, long-term therapy is required to maintain a response and prevent relapse.

Axial myoclonus in paraproteinemic polyneuropathy

“…The patient declined therapy with immunosuppressive agents. She was treated with plasmapheresis (once a week for the first 3 months as induction, followed by once a month as maintenance) 6, 11, 22. After 6 months, she reported an improvement in the myoclonus and a substantial reduction in the frequency and intensity of the axial jerks was shown by a repeated polygraphic examination performed with the patient sitting or lying in bed in various postures (Fig.…”

Patient demographics and health plan paid costs in chronic inflammatory demyelinating polyneuropathy

“…Prednisone and IVIg are considered first‐line therapies for CIDP . IVIg has been approved by the U.S. Food and Drug Administration for treatment of CIDP and was widely used off‐label before approval.…”

“…Prednisone and IVIg are considered first-line therapies for CIDP. [14][15][16][17] IVIg has been approved by the U.S. Food and Drug Administration for treatment of CIDP and was widely used off-label before approval. Not surprisingly, these were the most used immunotherapies in our cohort: 26% of patients had claims for IVIg, and 16% for prednisone.…”

Chronic inflammatory demyelinating polyneuropathy: diagnosis and management

“…These should only be prescribed by physicians with experience in immunosuppressive therapy and for patients who can be closely monitored [73].…”