Guidelines for diagnostic criteria and treatment of chronic inflammatory demyelinating
polyneuropathy (
CIDP) have been proposed by a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society, based on available evidence and expert consensus. These should prove practical for the clinical management of
CIDP.
Intravenous immunoglobulin followed by
corticosteroids should be considered as the initial treatment, however no clear second
drug of choice for patients who do not respond to the initial treatment is given. The author reports the long-term therapeutic efficacy of
ciclosporin for patients with
CIDP who did not show sustained improvement under
steroid therapy.
Ciclosporin should be tried for patients with intractable
CIDP who require repeated
intravenous immunoglobulin. An adequate initial dose of
ciclosporin is 3 mg/kg/day, with plasma trough concentrations between 100 and 150 ng/ml. If patients respond to
ciclosporin, remission can be maintained for 2 years, after which the dose can be slowly reduced over 1 year. Eventual withdrawal should be considered. This review proposes a treatment strategy that includes long-term maintenance
therapy for
CIDP based on published clinical trials and the author's clinical experience. Current concepts concerning the clinical spectrum of
CIDP and diagnostic approaches are also considered.