Chronic inflammatory demyelinating polyneuropathy: a treatment protocol proposal

Abstract

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.