Objective: To investigate the frequency and clinical correlates of anti-nerve autoantibodies in an unselected series of Italian patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) Methods: Sera from 276 CIDP patients fulfilling the EFNS/PNS criteria and included in the Italian CIDP database were examined for the presence of anti-nerve autoantibodies. Results were correlated with the clinical data collected in the database. Results: Anti-neurofascin155 (NF155) antibodies were found in 9/258 (3.5%) patients, anti-contactin1 (CNTN1) antibodies in 4/258 (1.6%) patients, and anti-contactin-associated protein1 (Caspr1) in 1/197 (0.5%) patients, while none had reactivity to gliomedin or neurofascin 186. Predominance of IgG4 isotype was present in 7of the 9 examined patients. Anti-NF155 patients more frequently had ataxia, tremor, and higher CSF protein levels than antibody-negative patients. Anti-CNTN1 patients more frequently had a GBS-like onset, pain, and ataxia and had more severe motor impairment at enrollment than antibody-negative patients. They more frequently received plasmapheresis, possibly reflecting a less satisfactory response to IVIg or steroids. IgM antibodies against one or more gangliosides were found in 6.5% of the patients (17/260) and were more frequently directed against GM1 (3.9%). They were frequently associated with a progressive course, with a multifocal sensorimotor phenotype and less frequent cranial nerve involvement and ataxia. Conclusions: Anti-paranodal and anti-ganglioside antibodies are infrequent in patients with CIDP but are associated with some typical clinical association supporting the hypothesis that CIDP might be a pathogenically heterogeneous syndrome possibly explaining the different clinical presentations.

Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database / Liberatore, G.; De Lorenzo, A.; Giannotta, C.; Manganelli, F.; Filosto, M.; Cosentino, G.; Cocito, D.; Briani, C.; Cortese, A.; Fazio, R.; Lauria, G.; Clerici, A. M.; Rosso, T.; Marfia, G. A.; Antonini, G.; Cavaletti, G.; Carpo, M.; Doneddu, P. E.; Spina, E.; Cotti Piccinelli, S.; Peci, E.; Querol, L.; Nobile-Orazio, E.. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-1874. - (2022). [10.1007/s10072-021-05811-0]

Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database

Manganelli F.;Spina E.;
2022

Abstract

Objective: To investigate the frequency and clinical correlates of anti-nerve autoantibodies in an unselected series of Italian patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) Methods: Sera from 276 CIDP patients fulfilling the EFNS/PNS criteria and included in the Italian CIDP database were examined for the presence of anti-nerve autoantibodies. Results were correlated with the clinical data collected in the database. Results: Anti-neurofascin155 (NF155) antibodies were found in 9/258 (3.5%) patients, anti-contactin1 (CNTN1) antibodies in 4/258 (1.6%) patients, and anti-contactin-associated protein1 (Caspr1) in 1/197 (0.5%) patients, while none had reactivity to gliomedin or neurofascin 186. Predominance of IgG4 isotype was present in 7of the 9 examined patients. Anti-NF155 patients more frequently had ataxia, tremor, and higher CSF protein levels than antibody-negative patients. Anti-CNTN1 patients more frequently had a GBS-like onset, pain, and ataxia and had more severe motor impairment at enrollment than antibody-negative patients. They more frequently received plasmapheresis, possibly reflecting a less satisfactory response to IVIg or steroids. IgM antibodies against one or more gangliosides were found in 6.5% of the patients (17/260) and were more frequently directed against GM1 (3.9%). They were frequently associated with a progressive course, with a multifocal sensorimotor phenotype and less frequent cranial nerve involvement and ataxia. Conclusions: Anti-paranodal and anti-ganglioside antibodies are infrequent in patients with CIDP but are associated with some typical clinical association supporting the hypothesis that CIDP might be a pathogenically heterogeneous syndrome possibly explaining the different clinical presentations.
2022
Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database / Liberatore, G.; De Lorenzo, A.; Giannotta, C.; Manganelli, F.; Filosto, M.; Cosentino, G.; Cocito, D.; Briani, C.; Cortese, A.; Fazio, R.; Lauria, G.; Clerici, A. M.; Rosso, T.; Marfia, G. A.; Antonini, G.; Cavaletti, G.; Carpo, M.; Doneddu, P. E.; Spina, E.; Cotti Piccinelli, S.; Peci, E.; Querol, L.; Nobile-Orazio, E.. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-1874. - (2022). [10.1007/s10072-021-05811-0]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11588/883190
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