[Chronic inflammatory demyelinating polyneuropathies: problem of the long-term therapeutic management]

No guideline is available for the long-term therapeutic management of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).

Efficacy of corticosteroids, intravenous immunoglobulins and plasma exchanges has been shown in trials conducted on short periods. Only experts'recommendations are available for management of patients after initiation of these treatments. For corticosteroids, decrease must be very slow on one or two years. With intravenous immunoglobulins, dose per cure must be diminished and then delay between cures extended. Other immunomodulators or immunosuppressors have been tested in CIDP in case of failure of standard treatment and if treatment dependence appears (cyclophosphamide, cyclosporine, azathioprine, mycophenolate, beta-1a interferon, rituximab, tacrolimus, etc.), but no controlled trials are yet available. Treatment of CIDP in the long term is very complex because this entity brings together very different patients. Thereby, in clinical and electophysiological series of the literature, therapeutic prognostic may depend on the delay between first symptoms and treatment, course of the disease, demyelinating pattern on EMG and development of axonal lesions.

Progress in the long-term treatment of CIDP depend on best diagnosis of the disease, fundamental research on pathophysiology to propose appropriate chemical molecules, and pursuit of trials with standard treatments on longer period and with others rhythm and way of administration. More recent treatments have to been tested in controlled trials too, in monotherapy or bi-therapy. Global cost in a patient treated for CIDP must be evaluated for each treatment. Multicentric approach of these questions will be recommended because of rarity of CIDP.