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

Authors: Liberatore, G; Lorenzo, A de; Giannotta, C; Manganelli, F; Filosto, M; Cosentino, G; Cocito, D; Briani, C; Cortese, A; Fazio, R; Lauria, G; Clerici, AM; Rosso, T; Marfia, GA; Antonini, G; Cavaletti, G; Carpo, M; Doneddu, PE; Spina, E; Cotti Piccinelli, S; Peci, E; Querol, L; Nobile-Orazio, E

Affiliations: Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, Manzoni 56, 20089, Rozzano, Italy; Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples “Federico II”, Naples, Italy; Center for Neuromuscular Diseases and Neuropathies, Unit of Neurology, ASST “Spedali Civili”, University of Brescia, Brescia, Italy; Department of Experimental BioMedicine and Clinical Neurosciences (BioNeC), University of Palermo, Palermo, Italy; IRCCS Foundation C. Mondino National Neurological Institute, Pavia, Italy. Presidio Sanitario Major, Istituti Clinici Scientifici Maugeri, Turin, Italy; Neurology Unit, Department of Neuroscience, University of Padua, Padua, Italy; Molecular Neurosciences, University College London, London, UK; Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milan, Italy; Unit of Neuroalgology, IRCCS Foundation “Carlo Besta” Neurological Institute, Milan, Italy; Department of Biomedical and Clinical Sciences “Luigi Sacco”, University of Milan, Milan, Italy; Neurology Unit, Circolo & Macchi Foundation Hospital, Insubria University, DBSV, Varese, Italy; ULSS2 Marca Trevigiana, UOC Neurologia-Castelfranco Veneto, Treviso, Italy; Dysimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy; Unit of Neuromuscular Diseases, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, “Sapienza” University of Rome, Sant’Andrea Hospital, Rome, Italy; School of Medicine and Surgery and Experimental Neurology Unit, University of Milano-Bicocca, Monza, Italy; ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy; Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples “Federico II”, Naples, Italy; Center for Neuromuscular Diseases and Neuropathies, Unit of Neurology, ASST “Spedali Civili”, University of Brescia, Brescia, Italy; Presidio Sanitario Major, Istituti Clinici Scientifici Maugeri, Turin, Italy; Neuromuscular Diseases Unit, Autoimmune Neurology, Neuromuscular Laboratory, Neurology Department, Hospital de la Santa Creu i Sant Pau, Institut de Recerca Biomèdica Sant Pau, Barcelona, Spain; Department of Medical Biotechnology and Translational Medicine, Milan University, Milan, Italy.

Publication: Neurological sciences ; 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.