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High-Dose Immunosuppression for Refractory CIDP?

The intensity of immunosuppression may be more important than the total cumulative dose in effectively treating CIDP.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy for which treatment with plasma exchange, intravenous immunoglobulin, or corticosteroids usually is beneficial. However, one third of CIDP patients do not respond to these conventional therapies, and many who respond initially relapse and develop substantial disability after several years. Compelling data suggest that both B and T cells mediate the immune attack on peripheral nerve, thus providing a biologic rationale for using more aggressive immunosuppressive regimens.

These researchers report their experience with high-dose cyclophosphamide (200 mg/kg over 4 days) in 4 patients with severe CIDP that was refractory to other therapies. All patients were disabled (Rankin scores of 4 or 5) and had failed numerous trials of standard therapies. Interestingly, 3 patients also had failed to improve after previous treatment with intravenous cyclophosphamide (1 g/m²).

All patients' Medical Research Council strength scores and Rankin disability scores improved; 2 patients improved by 3 Rankin grades. Febrile neutropenia and transient renal insufficiency occurred in 2 patients, and another developed line sepsis, but there was no long-term morbidity or mortality.

Comment: Current therapies for CIDP are inadequate for many patients. In this study, a 70-kg patient would have received 14 g of cyclophosphamide, only slightly more than the "immunomodulating" dose (1 g/m²) of 1.5 to 2.0 g administered as a pulse monthly for 6 months (a regimen that many centers already have adopted empirically). Therefore, the intensity of immunosuppression may be a more relevant factor than the total cumulative dose or the duration of therapy. The benefits of this approach must be weighed against high risks for systemic infection and for other serious complications. The risk-benefit balance may be in favor of high-dose immunosuppression if patients experience sustained remission and discontinue prednisone or other chronic immune (and potentially toxic) treatments.

— Kenneth C. Gorson, MD

Dr. Gorson is Associate Professor of Neurology, Tufts University School of Medicine, Boston.

Published in Journal Watch Neurology August 22, 2002

Citation(s):

Brannagan TH III et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology 2002 Jun 25; 58:1856-8.

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