Treatment of Chronic inflammatory demyelinating polyradiculoneuropathy

Early treatment to prevent loss of nerve axons in this rare autoimmune disease is recommended. Of course this is only possible if diagnosed early enough in disease course.

After treatment many people have a complete recovery and others have remaining numbness, weakness, tremors and fatigue which can lead to long-term poor health.

There are a variety of treatments for CIDP:

  • The main treatment for CIDP is steroids e.g. prednisone.
  • Immunosuppressive Drugs e.g. Azathioprine, Cyclo-phosphamide and Cyclosporin.
  • Plasma exchange called plasmapheresis
  • IVIg  - intravenous immune globulins - may be used.
  • Physiotherapy may help improve weak muscles and mobility of the joints.

IVIG and plasmapheresis have been proven to benefit CIDP sufferers in randomized, double-blind, placebo-controlled trials.

INFORMATION FOR DOCTORS 

 Excerpt. © Reprinted by permission. All rights reserved.

Clinical Scenario

Robert is a 55-year-old male who has been under your care for moderate obesity, type 2 diabetes, and hypertension. You last saw him 3 months ago for a change in his diabetes regimen.

At the current visit, you note that he is 10 lb heavier and appears to be depressed. He complains of weakness in the arms and legs plus chronic fatigue. He also complains of numbness in the hands and feet. On physical examination, he is weak proximally and distally in the arms and legs. No atrophy or fasciculations are noted. He has no reflexes in the upper or lower extremities. You detect distal loss of vibration and cold perception in the legs below the knees...

 Treatment text here published for CIDP is written by Per-Ola Kessman and Hakan Andersson.

First line
of treatment options for CIDP consists of three different types of anti-inflammatory therapies, steroids in tablet and / or tear-shaped, high-dose intravenous immunoglobulin (IVIG) in the drip and blood exchange (plasmapheresis). These three treatment options are tried and tested and considered to give equivalent results, at least in the short term.

Treatment with
plasmapheresis is now offered primarily for limited short periods associated with relapse if treatment with corticosteroids and IVIG are found to be effective.

The outcome
and safety profile of the three types of treatment varies among individuals, so it comes to try out the type of treatment that fits each person with CIDP best.

Treatment with
one or some of the above therapies in combination, usually result in a temporary and transient improvement in CIDP, which is why immunotherapy in these forms need to be sustained on a continuous basis. Nevertheless, it is not uncommon for people with CIDP to, over time, experience diminishing treatment effect and continued disease progression. In these cases, a concomitant therapy with anti-inflammatory chemotherapy is necessary to achieve long-term stabilization of CIDP symptoms.  Selection of exactly what form of chemotherapy to be added to the treatment  is done on a individual basis and after careful consideration of risk and profit.

It is likely, but currently unclear if the early initiation of chemotherapy in parallel with the initiation of immunotherapy with corticosteroids and / or IVIG may improve long-term prognosis of CIDP. Future treatment studies with newer generation chemotherapy suffer from fewer side effects, compared to the current ones, will determine if early adjunctive treatment with chemotherapy should be recommended for CIDP.

 Material has been developed by neurologists and other specialists and translated into English.

 

Swedish National Board of Health

Medline Chronic relapsing (dysimmune) polyneuropathy: pathogenesis and treatment.

Ninds Information Page on Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Phoenix Neurological Associates

Pubmed Can sural nerve biopsy be used to orientate the treatment?

IN Group

Chronic inflammatory demyelinating polyneuropathies: Current treatment strategies

 

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