Interferon Alpha May Benefit Cidp Patients Refractory To Standard Therapies: Description Of Long‐Term Results On 12 Patients

Abstract

Although most patients affected by CIDP improve with Corticosteroids, Intravenous Immunoglobulins or Plasma‐Exchange, about 20–30% show poor or no response and 7–10% die due to the disease. We describe 12 CIDP patients unresponsive to standard therapies who were treated with Interferon alpha. Follow‐up period ranged from 4 months to 8 years. Six patients showed a marked and sustained improvement: three recovered completely; the others, who had a severe quadriparesis (Rankin 5) before treatment disclosed a score 2–3 at last follow up. Beginning of improvement was observed within 2–4 days since starting therapy in most patients. In the remaining 6 patients no improvement was observed and two of them died with quadriplegia and respiratory failure. No significant adverse effects were observed. Over the last decade Interferons have been used in the treatment of CIDP. Some authors reported no significant improvement (Hadden et al., Neurology 1999, 53:57; Kuntzer et al., Neurology 1999, 53:1364) while others documented a clear benefit (Engel and Adornato, Neurology 1992, 42:467; Sabatelli, J Neurol Neurosurg Psych 1995, 58:638; Gorson et al., Neurology 1997, 48:777; Gorson et al., Neurology 1998, 50:84; Harada et al., 2000, MN:295; Ueda et al., Rinsho Shink 2000, 40:155). The ever present problem in clinical studies on CIDP, selection bias, may explain this discrepancy. However, the type of Interferon employed may be crucial because only patients receiving IFN alpha improved. Our data show that 50% of patients treated with IFN alpha disclose a significant improvement thus suggesting that this therapy may represent an effective option in otherwise intractable CIDP patients.