Treatment of Chronic Inflammatory Demyelinating Polyneuropathy With High-Dose Intermittent Intravenous Methylprednisolone

ArticleinJAMA Neurology 62(2):249-54 · March 2005with60 Reads
DOI: 10.1001/archneur.62.2.249 · Source: PubMed
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) causes progressive disability due to weakness but responds to immunomodulating medication, including oral prednisone and intravenous (IV) immunoglobulin (IVIg). However, there is no consensus on initial therapy, and both of these treatments have drawbacks with long-term treatment. To review the efficacy and safety of high-dose, intermittent IV methylprednisolone (IVMP) as initial and long-term maintenance therapy for patients with CIDP. A retrospective medical record review between 1992 and 2003 of outcomes in CIDP, comparing patients in 3 cohorts depending on whether their primary treatment was IVMP, IVIg, or oral immunosuppression with prednisone or cyclosporine. Washington University Neuromuscular Disease Center (St Louis, Mo), outpatient and inpatient records. Patients with clinical and electrophysiologic evidence of CIDP were identified. Of 57 patients, 39 had sufficient data for full analysis. Quantitative muscle testing with a handheld dynamometer. Medication profiles and adverse effects were also recorded. There was no significant difference in the mean improvement in quantitative muscle testing at 6 months or at the last clinic visit (an average of 4.5 years later) among the 3 groups. Fewer patients treated with oral immunosuppression improved at 6 months, but at the last visit, 81% to 88% improved in all 3 groups. Less weight gain and fewer cushingoid features affected patients treated with IVMP (19%) compared with patients treated with oral prednisone (58%). Treatment of patients with CIDP using high-dose intermittent IVMP results in improved strength equal to that with IVIg and oral prednisone. The frequency of occurrences of weight gain and cushingoid features with IVMP is less than that with oral prednisone. Intravenous methylprednisolone should be considered for initial and long-term therapy in CIDP when patients have disability due to weakness.
    • "Clinical improvement was measured with quantitative muscle test. Although the tests used and the regimens given were different from ours, they reported a similar remission rate [5]. Nobile-Orazio et al. compared efficacy of IVIG and intravenous methylprednisolone in 45 CIDP patients [9]. "
    [Show abstract] [Hide abstract] ABSTRACT: Although various modalities of treatment of chronic inflammatory demyelinating polyradiculopathy (CIDP) there are not any treatment protocol agreed. We retrospectively evaluated the 20 CIDP patients (14 male, 6 female). Five patients were excluded from the study because they could not continue their treatments due to various problems during the treatment. The remaining 15 patients treated with monthly high dose intravenous methyl prednisolone for five years (IVMP) and followed up for 10 years. The mean age of the patients was 48.1±14.6 years. The mean duration of disease was 6.8±3.1 years. We were found statistically significant difference between the pre-treatment and sixth month modified Rankin scores (p<0.001). Similarly, significant improvement was observed at the end of first, fourth and fifth years of treatment. Statistically significant difference was found between baseline and tenth year modified Rankin scores of 12 patients who were treated with only IVMP during 10 years follow-up. Long-term monthly IVMP pulse therapy seems to be very effective in the treatment of CIDP.
    Full-text · Article · Mar 2014
    • "Adverse effects were similar between the groups, but one wonders whether side effects may have increased in the prednisone group with longer exposure [Gorson, 2010]. A retrospective study has suggested that intravenous , high-dose (1 g) methylprednisolone, administered daily for 3–5 days followed by a weekly regimen for 4–8 weeks, and then monthly as dictated by clinical course, was effective in 13 of 16 patients at 6-month follow up [Lopate et al. 2005]. If confirmed in larger, randomized, controlled trials , this approach may provide an effective alternative to daily oral prednisone with fewer adverse effects. "
    [Show abstract] [Hide abstract] ABSTRACT: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune mediated disorder of the peripheral nervous system with clinical features that include weakness, sensory loss, imbalance, pain and impaired ambulation which may lead to substantial disability. This review highlights current treatment strategies for CIDP, how best to utilize proven therapies such as intravenous immunoglobulin, oral prednisone, pulse dexamethasone, and plasma exchange, and when and how to use alternative immunosuppressive agents when first-line therapies are ineffective or poorly tolerated.
    Full-text · Article · Nov 2012
    • "The typical starting dose of prednisone is 1—1.5 mg/kg/day for 2—4 weeks [Dyck et al. 1982; Dalakas and Engel, 1981; Prineas and McLeod, 1976]. Intravenous steroid pulse therapy is also used in more severe cases [Lopate et al. 2005; Molenaar et al. 1997]. At lower dosage below 1 mg/kg body weight, glucocorticoids inhibit inflammation by activating the cytosolic glucocorticoid receptor. "
    [Show abstract] [Hide abstract] ABSTRACT: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated polyradiculoneuritis that is progressive or relapsing over a period of at least 8 weeks. Although the exact pathogenesis is unclear, it is thought to be mediated by both cellular and humoral immune reactions directed against the peripheral nerve myelin or axon. CIDP also involves spinal nerve roots. Early medical treatment of CIDP is important to prevent axonal loss. Only three treatment regimens for CIDP have demonstrated benefit in randomized, controlled studies: corticosteroids, plasma exchange, and intravenous immunoglobulins (IVIg). Approximately 25% of patients respond inadequately to corticosteroids, plasma exchange or IVIg. Large placebo-controlled trials with alternative immunosuppressive compounds, e.g. mycophenolate mofetil, cyclosporine, cyclophosphamide, or monoclonal antibodies, are lacking.
    Article · May 2011
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