TY - JOUR
T1 - Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids
AU - Muley, Suraj Ashok
AU - Kelkar, Praful
AU - Parry, Gareth J.
PY - 2008/11
Y1 - 2008/11
N2 - 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. Design: Open-label prospective study. Setting: University of Minnesota Neuropathy Center, Minneapolis. Patients: Ten patients (3 women and 7 men) with CIDP followed up for at least 22 months. Main Outcome Measures: 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.
AB - 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. Design: Open-label prospective study. Setting: University of Minnesota Neuropathy Center, Minneapolis. Patients: Ten patients (3 women and 7 men) with CIDP followed up for at least 22 months. Main Outcome Measures: 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.
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U2 - 10.1001/archneur.65.11.1460
DO - 10.1001/archneur.65.11.1460
M3 - Article
C2 - 19001164
AN - SCOPUS:55949098762
SN - 0003-9942
VL - 65
SP - 1460
EP - 1464
JO - Archives of Neurology
JF - Archives of Neurology
IS - 11
ER -