Early and effective treatment of chronic inflammatory demyelinating
polyneuropathy (
CIDP) is important to minimize axonal degeneration that occurs secondary to
demyelination. The disease course is invariably chronic, so long-term treatment is often required, and adverse effects and costs are important considerations in devising a treatment plan.
CIDP responds to
prednisone, but long-term treatment can result in significant adverse effects.
Azathioprine,
mycophenolate mofetil, and
cyclosporine can be used as
steroid-sparing agents and may facilitate more rapid and successful tapering of
prednisone.
Intravenous immunoglobulin (
IVIg) and
plasma exchange are also effective in the treatment of
CIDP and can be used in patients who are unresponsive to
prednisone or develop
steroid-related adverse effects.
IVIg may also be used as a first-line treatment, but its cost can be a limiting factor. A few uncontrolled studies have suggested that pulsed weekly
methylprednisolone is both effective and well tolerated in the long-term treatment of
CIDP. Treatments based on
rituximab or
cyclophosphamide have also been used in resistant disease. Variants of
CIDP have been described on the basis of their association with specific
antibodies or
immunoglobulins and their response to specific immunomodulatory treatments. Multifocal motor neuropathy with conduction block responds to
IVIg in the majority of patients. However, weakness may slowly worsen over time, and some patients become unresponsive. Anecdotal reports suggest that
rituximab may be useful in patients who develop progressive disease. Placebo-controlled trials in anti-
myelin-associated glycoprotein neuropathy suggest that
rituximab is effective and, with a combination of
prednisone and
cyclophosphamide,
numbness and strength may improve. Other treatments that may be effective include
plasma exchange and
IVIg. Treatment is generally started with
prednisone,
IVIg, or
plasma exchange.
Rituximab and
cyclophosphamide are used only in progressive, treatment-resistant disease because of the potential for serious adverse effects.