Treatment Recommendations

Carol L. Koski, M.D., Principal Author

Treatment recommendations have been proposed by a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. (Joint Task Force, 2005)

The joint task force recommends:

1. Intravenous immunoglobulin (IVIG) or corticosteroids should be considered first line in sensory and motor CIDP;
2. IVIG should be considered as an initial treatment in pure motor CIDP;
3. If IVIG and corticosteroids are ineffective, plasma exchange should be considered;
4. If the response after 3 months is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulating drug should be considered; and
5. Symptomatic treatment and multidisciplinary management should be considered.

The EFNS/PNS Guidelines as noted above suggest that IVIG should be considered as an initial treatment in pure motor CIDP. Pure motor CIDP is clinically distinct from many of the other demyelinating neuropathies.

The guidelines above address the recommendation of prescribing plasma exchange therapy when IVIG or prednisone therapies have not proven to be effective. Most researchers and many practicing neurologists will continue the trial of initial therapy of IVIG or prednisone for at least one month, sometimes two months or even longer, depending on the patient and symptoms, before initiating plasma exchange therapy. Still others will prescribe plasma exchange for patients while the patient is still on initial therapy.

Also, the guidelines recommend combination treatment or adding other drugs to the initial regimen. The addition of 30 mg to 40 mg of prednisone daily reduced the frequency of repeated IVIG therapy. (Hahn, 1996); the addition of cyclophosphamide and cyclosporin has been of limited benefit in some patients. (Gorson, 1997)