TY - JOUR
T1 - Clinical relevance of distinguishing autoimmune nodopathies from CIDP
T2 - Longitudinal assessment in a large cohort
AU - Broers, Merel C.
AU - Wieske, Luuk
AU - Erdag, Ece
AU - Gürlek, Cemre
AU - Bunschoten, Carina
AU - van Doorn, Pieter A.
AU - Eftimov, Filip
AU - Kuitwaard, Krista
AU - de Vries, Juna M.
AU - de Wit, Marie-Claire Y.
AU - Nagtzaam, Mariska Mp
AU - Franken, Suzanne C.
AU - Zhu, Louisa
AU - Paunovic, Manuela
AU - de Wit, Maurice
AU - Schreurs, Marco W. J.
AU - Lleixà, Cinta
AU - Martín-Aguilar, Lorena
AU - Pascual-Goñi, Elba
AU - Querol, Luis
AU - Jacobs, Bart C.
AU - Huizinga, Ruth
AU - Titulaer, Maarten J.
N1 - Publisher Copyright: © 2024 BMJ Publishing Group. All rights reserved.
PY - 2024/1/1
Y1 - 2024/1/1
N2 - Background: The aim of this study was to determine treatment response and whether it is associated with antibody titre change in patients with autoimmune nodopathy (AN) previously diagnosed as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and to compare clinical features and treatment response between AN and CIDP. Methods: Serum IgG antibodies to neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (CASPR1) were detected with cell-based assays in patients diagnosed with CIDP. Clinical improvement was determined using the modified Rankin scale, need for alternative and/or additional treatments and assessment of the treating neurologist. Results: We studied 401 patients diagnosed with CIDP and identified 21 patients with AN (10 anti-NF155, 6 anti-CNTN1, 4 anti-CASPR1 and 1 anti-NF155/anti-CASPR1 double positive). In patients with AN ataxia (68% vs 28%, p=0.001), cranial nerve involvement (34% vs 11%, p=0.012) and autonomic symptoms (47% vs 22%, p=0.025) were more frequently reported; patients with AN improved less often after intravenous immunoglobulin treatment (39% vs 80%, p=0.002) and required additional/alternative treatments more frequently (84% vs 34%, p<0.001), compared with patients with CIDP. Antibody titres decreased or became negative in patients improving on treatment. Treatment withdrawal was associated with a titre increase and clinical deterioration in four patients. Conclusions: Distinguishing CIDP from AN is important, as patients with AN need a different treatment approach. Improvement and relapses were associated with changes in antibody titres, supporting the pathogenicity of these antibodies.
AB - Background: The aim of this study was to determine treatment response and whether it is associated with antibody titre change in patients with autoimmune nodopathy (AN) previously diagnosed as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and to compare clinical features and treatment response between AN and CIDP. Methods: Serum IgG antibodies to neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (CASPR1) were detected with cell-based assays in patients diagnosed with CIDP. Clinical improvement was determined using the modified Rankin scale, need for alternative and/or additional treatments and assessment of the treating neurologist. Results: We studied 401 patients diagnosed with CIDP and identified 21 patients with AN (10 anti-NF155, 6 anti-CNTN1, 4 anti-CASPR1 and 1 anti-NF155/anti-CASPR1 double positive). In patients with AN ataxia (68% vs 28%, p=0.001), cranial nerve involvement (34% vs 11%, p=0.012) and autonomic symptoms (47% vs 22%, p=0.025) were more frequently reported; patients with AN improved less often after intravenous immunoglobulin treatment (39% vs 80%, p=0.002) and required additional/alternative treatments more frequently (84% vs 34%, p<0.001), compared with patients with CIDP. Antibody titres decreased or became negative in patients improving on treatment. Treatment withdrawal was associated with a titre increase and clinical deterioration in four patients. Conclusions: Distinguishing CIDP from AN is important, as patients with AN need a different treatment approach. Improvement and relapses were associated with changes in antibody titres, supporting the pathogenicity of these antibodies.
KW - clinical neurology
KW - neuroimmunology
KW - peripheral neuropathology
UR - http://www.scopus.com/inward/record.url?scp=85175814340&partnerID=8YFLogxK
U2 - https://doi.org/10.1136/jnnp-2023-331378
DO - https://doi.org/10.1136/jnnp-2023-331378
M3 - Article
C2 - 37879898
SN - 0022-3050
VL - 95
SP - 52
EP - 60
JO - Journal of Neurology, Neurosurgery and Psychiatry
JF - Journal of Neurology, Neurosurgery and Psychiatry
IS - 1
M1 - jnnp-2023-331378
ER -