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Long-Term Prognosis Of CIDP: A Study of 49 Patients

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In 1975 Dyck et al. (Mayo Clin Proc. 1975, 50:621) reported that 10% of their patients with CIDP died, 28% were confined to wheelchair/bed and 8% were ambulatory and not able to work. More recent papers (Bouchard et al., Neurology 1999, 52:498; Gorson et al., Neurology 1997, 48:321) have reported quite similar data: 27–29% of treated patients need assistance for most activities and 7–10% of patients die. These findings are in contrast with the progressive expansion of therapeutic repertoire observed over the last 25 years. We describe the outcome of 49 patients affected by CIDP observed in our Institute from 1986 to 2000. All patients underwent extensive electrophysiological examination. Sural nerve biopsy was carried out in 45/49 patients. Selection criteria included clear-cut evidence of conduction blocks or demyelination at pathological examination. Patients were treated with corticosteroids, IVIg, and PE. Patients on refractory to standard therapies received azathioprine or Interferon alpha. Taken as a whole, outcome was good in 84% of our patients: 33 (67%) achieved clinical remission and 8 (17%) improved significantly. 12% of our patients were unresponsive to all treatments. 4% died as the result of progression of the disease to severe quadriplegia with respiratory failure. Patients achieving recovery were separated in two distinct groups: patients showing a remission period after treatment withdrawal (16 patients) and patients needing permanent therapies to maintain improvement (17 patients). In the former group, 8 patients had a monophasic course with a follow-up of 6 months to 9 years (mean 47 months); 8 patients showed a relapsing-remitting course with a drug-free period ranging from 6 months to 20 years, 50% reaching a remission period of at least 5 years. Regarding the latter group, clinical remission while taking therapies lasted 3 months to 4 years. In conclusion, we found that prognosis of CIDP is better than reported in the literature. Selection criteria and different therapeutic approach may explain such discrepancy.
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Document Type: Abstract

Affiliations: Neurological Institute, Catholic University, Rome.

Publication date: March 1, 2001

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