TY - JOUR
T1 - Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy
AU - Bunschoten, Carina
AU - Jacobs, Bart C.
AU - Van den Bergh, Peter Y.K.
AU - Cornblath, David R.
AU - van Doorn, Pieter A.
N1 - Funding Information:
PAvD reports grants from Sanquin, Prinses Beatrix Spierfonds, Baxalta, Grifols. He is on the trial steering committee of Octapharma and is a consultant for Kedrion, CSL Behring, and Hansa, outside of the submitted work. BCJ receives grants from Baxalta, Grifols, CSL-Behring, Annexon, Prinses-Beatrix Spierfonds, and GBS-CIDP Foundation International, and is on the Global Medical Advisory Board of the GBS CIDP Foundation International. PYKVdB is a consultant for Pfizer, Genzyme, CSL Behring, LFB France, Natus, UCB Pharma and Alnylam. DRC is a consultant for Annexon Biosciences, Argenx BVBA, Biotest Pharmaceuticals, Inc, Cigna Health Management, CSL Behring, DP Clinical, Inc., Grifols SA, Hansa Medical, Merrimack Pharmaceuticals, Neurocrine Biosciences, Octapharma AG, Pharnext SAS, Sun Pharmaceuticals, and Syntimmune. He sits on Data Safety Monitoring Boards for Momenta Pharma, Pfizer, PledPharma, Technology Licensing for the Total Neuropathy Score to AstraZeneca Pharmaceuticals, Calithera Biosciences, Genentech, Neurocrine Biosciences, Merrimack Pharmaceuticals, and Seattle Genetics. He is on the Global Medical Advisory Board of the GBS CIDP Foundation International. He is Editor-in-Chief of the Journal of the Peripheral Nervous System . CB declares no competing interests.
Publisher Copyright:
© 2019 Elsevier Ltd
PY - 2019/8
Y1 - 2019/8
N2 - Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. Nerve conduction studies are considered essential for a definite diagnosis, but poor performance and misinterpretation of the results frequently leads to misdiagnosis. Nerve ultrasound and MRI could be helpful in diagnosis. Whereas typical CIDP is relatively easy to diagnose, atypical variants with distinct phenotypes can be a diagnostic challenge. Intravenous or subcutaneous immunoglobulin, corticosteroids, and plasma exchange are effective treatments, but maintenance treatments are often required for years, and treatment regimens require careful and regular adjustments to avoid undertreatment or overtreatment. Patients who do not improve, or insufficiently improve after treatment, might have specific characteristics related to a distinct disease mechanism caused by immunoglobulin G4 antibodies to nodal or paranodal proteins, and could require alternative treatments. Future studies should focus on curative and individualised treatment regimens to improve the patient's condition and to prevent further nerve damage.
AB - Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. Nerve conduction studies are considered essential for a definite diagnosis, but poor performance and misinterpretation of the results frequently leads to misdiagnosis. Nerve ultrasound and MRI could be helpful in diagnosis. Whereas typical CIDP is relatively easy to diagnose, atypical variants with distinct phenotypes can be a diagnostic challenge. Intravenous or subcutaneous immunoglobulin, corticosteroids, and plasma exchange are effective treatments, but maintenance treatments are often required for years, and treatment regimens require careful and regular adjustments to avoid undertreatment or overtreatment. Patients who do not improve, or insufficiently improve after treatment, might have specific characteristics related to a distinct disease mechanism caused by immunoglobulin G4 antibodies to nodal or paranodal proteins, and could require alternative treatments. Future studies should focus on curative and individualised treatment regimens to improve the patient's condition and to prevent further nerve damage.
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U2 - 10.1016/S1474-4422(19)30144-9
DO - 10.1016/S1474-4422(19)30144-9
M3 - Review article
C2 - 31076244
AN - SCOPUS:85068517684
SN - 1474-4422
VL - 18
SP - 784
EP - 794
JO - The Lancet Neurology
JF - The Lancet Neurology
IS - 8
ER -