Submitted by: Pritikanta Paul, MD
Edited by: Milvia Y. Pleitez, MD
Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision [published online ahead of print, 2021 Jun 4]. J Peripher Nerv Syst. 2021;10.1111/jns.12455. doi:10.1111/jns.12455
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) diagnosis is based on clinical, electrophysiological, and other laboratory investigations. The European Federation of Neurological Societies/Peripheral Nerve Society diagnostic criteria (2010) for CIDP has been found to have high sensitivity and specificity. The second revision has been recently published with goals of improving diagnostic accuracy and treatment outcome.
Task Force members used 12 Population/Intervention/Comparison/Outcome (PICO) formatted research questions addressing diagnosis and treatment and reviewed literature between June 2018 and July 2019.
The revisions included revising clinical criteria into ‘Typical CIDP’ and ‘CIDP variants’, the later replacing ‘atypical CIDP’ since patients with ‘atypical CIDP’ (distal CIDP, focal CIDP, mutifocal CIDP, motor CIDP, sensory CIDP) have well-described clinical and electrophysiological phenotypes. Chronic immune sensory polyradiculopathy (CISP) thought to similar to sensory CIDP but differentiated by having normal nerve conduction studies (NCS), was not included as a CIDP variant despite being responsive to immunotherapy. Patients often fulfilling the 2010 diagnostic criteria of CIDP but with nodal/paranodal antigens were not included in the CIDP variants but proposed to be classified them as autoimmune nodopathies as they have distinct phenotypes and respond poorly to intravenous immunoglobulins (IVIg).
The levels of diagnostic certainty as well as electrodiagnostic certainty was reduced to only two levels (CIDP and possible CIDP) as diagnostic accuracy of criteria for probable and definite CIDP did not vary significantly. Additionally, sensory conduction studies were included in electrophysiology criteria not included in previous version. Ultrasound (US) imaging was suggested as part of supportive criteria for patients fulfilling criteria for possible CIDP but not CIDP.
IVIg or corticosteroids remain strong recommendation for treatment induction in CIDP or CIDP variants with plasma exchange being next choice if IVIG or steroids are ineffective. There was no preference for either IVIg or subcutaneous immunoglobulin (SCIg) for maintenance treatment in CIDP.
In absence of a single specific biomarker, diagnosis of CIDP can be challenging and sometimes missed. The revised guidelines will further help in accurate diagnosis and better treatment outcomes.
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.Van den Bergh PY, Hadden RD, Bouche P, Cornblath DR, Hahn A, Illa I, Koski CL, Léger JM, Nobile-Orazio E, Pollard J, Sommer C, van Doorn PA, van Schaik IN; European Federation of Neurological Societies; Peripheral Nerve Society.Eur J Neurol. 2010 Mar;17(3):356-63.