Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids.
BACKGROUND
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy that responds to various immunosuppressive treatments. Oral daily prednisone therapy is effective and inexpensive, but the long-term treatment that is usually necessary leads to serious adverse effects. Consequently, intravenous immunoglobulin and plasma exchange have been widely used to treat CIDP, making treatment expensive and inconvenient. A steroid regimen that reduces adverse effects but preserves efficacy would simplify treatment. Pulsed steroids have nongenomic actions not seen with low-dose steroids, including rapid inhibition of arachidonic acid release and of calcium and sodium cycling across plasma membranes of immune cells.


OBJECTIVE
To study the efficacy, safety, and tolerability of pulsed oral methylprednisolone therapy in patients with CIDP.


METHODS
Open-label prospective study.


METHODS
University of Minnesota Neuropathy Center, Minneapolis.


METHODS
Ten patients (3 women and 7 men) with CIDP followed up for at least 22 months.


METHODS
Neuromuscular score and Inflammatory Neuropathy Cause and Treatment (INCAT) disability score were used as outcome measures for efficacy; weight, blood pressure, changes in bone density, and steroid-related adverse effect questionnaire were used as outcome measures for safety.


RESULTS
This steroid regimen leads to significant improvement in weakness and disability in all patients treated and to off-treatment remission in 60% of patients. Treatment was fairly well tolerated, and only 1 patient discontinued treatment because of adverse effects. Steroid-induced osteoporosis remained a problem, especially in older patients.


CONCLUSIONS
Pulsed oral methylprednisolone may be efficacious in the long-term treatment of CIDP and is relatively well tolerated. Remission can be induced in most patients, especially those with a shorter duration of disease.
Muley
Suraj Ashok
SA
Department of Neurology, University of Minnesota, Minneapolis, MN 55455, USA. muley001@umn.edu
Kelkar
Praful
P
Parry
Gareth J
GJ
D000284:Administration, Oral; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004185:Disability Evaluation; D004334:Drug Administration Schedule; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D008875:Middle Aged; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000188:drug therapy* / Q000503:physiopathology; D011446:Prospective Studies; D012074:Remission Induction; D016896:Treatment Outcome
D005938:Glucocorticoids; D008775:Methylprednisolone