European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society — First Revision

TLDR

Previous consensus guidelines on the definition, investigation, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy were published in the European Journal of Neurology and the Journal of the Peripheral Nervous System. The aim of this task force was to revise those existing guidelines. Disease experts, including a patient representative, reviewed MEDLINE and Cochrane Systematic Reviews from August 2004 to July 2009 and iteratively agreed on statements. The Task Force issued Good Practice Points for diagnostic criteria and investigations, and recommended IVIg (Level A) or corticosteroids (Level C) for sensory and motor CIDP, IVIg as first‑line for pure motor CIDP, plasma exchange if initial therapies fail, and combination or immunosuppressive/additive treatments when responses are inadequate or maintenance doses are high, alongside symptomatic and multidisciplinary care.

Abstract

Background: Consensus guidelines on the definition, investigation, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been previously published in European Journal of Neurology and Journal of the Peripheral Nervous System . Objectives: To revise these guidelines. Methods: Disease experts, including a representative of patients, considered references retrieved from MEDLINE and Cochrane Systematic Reviews published between August 2004 and July 2009 and prepared statements that were agreed in an iterative fashion. Recommendations: The Task Force agreed on Good Practice Points to define clinical and electrophysiological diagnostic criteria for CIDP with or without concomitant diseases and investigations to be considered. The principal treatment recommendations were: (i) intravenous immunoglobulin (IVIg) (Recommendation Level A) or corticosteroids (Recommendation Level C) should be considered in sensory and motor CIDP; (ii) IVIg should be considered as the initial treatment in pure motor CIDP (Good Practice Point); (iii) if IVIg and corticosteroids are ineffective, plasma exchange (PE) should be considered (Recommendation Level A); (iv) if the response is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulatory drug should be considered (Good Practice Point); (v) symptomatic treatment and multidisciplinary management should be considered (Good Practice Point).