دورية أكاديمية
Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype
| العنوان: | Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype |
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| المؤلفون: | Cortese A., Lombardi R., Briani C., Callegari I., Benedetti L., Manganelli F., Luigetti M., Ferrari S., Clerici A. M., Marfia G. A., Rigamonti A., Carpo M., Fazio R., Corbo M., Mazzeo A., Giannini F., Cosentino G., Zardini E., Curro R., Gastaldi M., Vegezzi E., Alfonsi E., Berardinelli A., Kouton L., Manso C., Giannotta C., Doneddu P., Dacci P., Piccolo L., Ruiz M., Salvalaggio A., De Michelis C., Spina E., Topa A., Bisogni G., Romano A., Mariotto S., Mataluni G., Cerri F., Stancanelli C., Sabatelli M., Schenone A., Marchioni E., Lauria G., Nobile-Orazio E., Devaux J., Franciotta D. |
| المساهمون: | Cortese, A., Lombardi, R., Briani, C., Callegari, I., Benedetti, L., Manganelli, F., Luigetti, M., Ferrari, S., Clerici, A. M., Marfia, G. A., Rigamonti, A., Carpo, M., Fazio, R., Corbo, M., Mazzeo, A., Giannini, F., Cosentino, G., Zardini, E., Curro, R., Gastaldi, M., Vegezzi, E., Alfonsi, E., Berardinelli, A., Kouton, L., Manso, C., Giannotta, C., Doneddu, P., Dacci, P., Piccolo, L., Ruiz, M., Salvalaggio, A., De Michelis, C., Spina, E., Topa, A., Bisogni, G., Romano, A., Mariotto, S., Mataluni, G., Cerri, F., Stancanelli, C., Sabatelli, M., Schenone, A., Marchioni, E., Lauria, G., Nobile-Orazio, E., Devaux, J., Franciotta, D. |
| بيانات النشر: | NLM (Medline) |
| سنة النشر: | 2020 |
| المجموعة: | Padua Research Archive (IRIS - Università degli Studi di Padova) |
| مصطلحات موضوعية: | neurofascin, neuropathis, paranode |
| الوصف: | Objective: To assess the prevalence and isotypes of anti-nodal/paranodal antibodies to nodal/paranodal proteins in a large chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) cohort, compare clinical features in seronegative vs seropositive patients, and gather evidence of their isotype-specific pathogenic role. Methods: Antibodies to neurofascin-155 (Nfasc155), neurofascin-140/186 (Nfasc140/186), contactin-1 (CNTN1), and contactin-associated protein 1 (Caspr1) were detected with ELISA and/or cell-based assay. Antibody pathogenicity was tested by immunohistochemistry on skin biopsy, intraneural injection, and cell aggregation assay. Results: Of 342 patients with CIDP, 19 (5.5%) had antibodies against Nfasc155 (n = 9), Nfasc140/186 and Nfasc155 (n = 1), CNTN1 (n = 3), and Caspr1 (n = 6). Antibodies were absent from healthy and disease controls, including neuropathies of different causes, and were mostly detected in patients with European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) definite CIDP (n = 18). Predominant antibody isotypes were immunoglobulin G (IgG)4 (n = 13), IgG3 (n = 2), IgG1 (n = 2), or undetectable (n = 2). IgG4 antibody-associated phenotypes included onset before 30 years, severe neuropathy, subacute onset, tremor, sensory ataxia, and poor response to intravenous immunoglobulin (IVIG). Immunosuppressive treatments, including rituximab, cyclophosphamide, and methotrexate, proved effective if started early in IVIG-resistant IgG4-seropositive cases. Five patients with an IgG1, IgG3, or undetectable isotype showed clinical features indistinguishable from seronegative patients, including good response to IVIG. IgG4 autoantibodies were associated with morphological changes at paranodes in patients' skin biopsies. We also provided preliminary evidence from a single patient about the pathogenicity of anti-Caspr1 IgG4, showing their ability to penetrate paranodal regions and disrupt the integrity of the Nfasc155/CNTN1/Caspr1 complex. Conclusions: Our findings ... |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| العلاقة: | info:eu-repo/semantics/altIdentifier/pmid/31753915; info:eu-repo/semantics/altIdentifier/wos/WOS:000530690100007; volume:7; issue:1; firstpage:e639; journal:NEUROLOGY® NEUROIMMUNOLOGY & NEUROINFLAMMATION; http://hdl.handle.net/11577/3322617Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85075510481 |
| DOI: | 10.1212/NXI.0000000000000639 |
| الإتاحة: | https://doi.org/10.1212/NXI.0000000000000639Test http://hdl.handle.net/11577/3322617Test |
| حقوق: | info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.D6BC88AA |
| قاعدة البيانات: | BASE |
| DOI: | 10.1212/NXI.0000000000000639 |
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