P.88 Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated peripheral nerve disorder characterized by muscle weakness and sensory deficits evolving over a period of more than 8 weeks, with electrodiagnostic evidence of peripheral nerve demyelination. Considering the rarity of the disease and the ongoing evolution in diagnosis and treatment, little is known about the long-term course of these patients. We describe the treatment and evolution over a period of more than 15 years in 29 patients. Patients had a median follow-up time of 16.5 years. All patients were treated with at least one treatment modality (corticosteroids, immunoglobulins, plasmapheresis, or other immunosuppressive treatments). 38% of patients were in remission at the end of follow up, 49% of patients were still treatment-dependent, and 17% showed a progressive course regardless of treatment. At 15-year follow up visit, 59% of patients had a better or unchanged disability status compared to time of diagnosis, and 41% was more disabled. Longer treatment delay was correlated with unfavourable clinical evolution over 15 years. Robust data on long-term evolution of CIDP patients is still lacking. Our study indicates that an important proportion of patients seem to develop progressive disability over time. Globally, there was no significant difference in disability between diagnosis and 15 years, but there was a trend to worsening, especially for patients with long-term treatment dependence or lack of sustained response. Further research on determining factors of long-term prognosis of CIDP would be desirable. Treatment delay is associated with worse outcome and should therefore be avoided.