Anti-MAG antibodies in 202 patients: Clinicopathological and therapeutic features - 27/03/18
Résumé |
The purpose of this study was to assess the clinicopathological and therapeutic features of patients with low (≥1000–<10 000 Bühlmann® Titre Units) (BTU), medium (10 000–70 000) or high (≥70 000) anti-Myelin-Associated Glycoprotein (MAG) antibody titres.
Methods |
We retrospectively and prospectively analysed standardized report forms and medical records of two hundred and two patients from fourteen neuromuscular centres.
Results |
Mean age at onset and mean time between symptom onset to last follow-up were respectively 62.6 years (25–91.4) and 8.4 years (0.3–33.3). Anti-MAG antibody titres at diagnosis were low, medium or high in 11, 51 and 38% of patients. Patients presented with monoclonal gammopathy of undetermined significance (MGUS) in 68% of cases. About 17% of patients presented with “atypical” clinical phenotype independently of anti-MAG titres, including acute or chronic sensorimotor polyradiculoneuropathies (12.4%), and asymmetric or multifocal neuropathy (3%). At the most severe disease stage, 22.4% of patients were significantly disabled. Seventy eight percent of patients received immunotherapies. Transient clinical worsening was observed in 12% of patients treated with rituximab (11/92). Stabilisation after rituximab treatment during the 7–12-month follow-up period was observed in 29% of patients. Clinical response to rituximab during the 6-month and/or 7–12-month follow-up period was observed in 31.5% of patients, and correlated with anti-MAG titre ≥10 000BTU. Our study highlights the extended clinical spectrum of patients with anti-MAG neuropathy, which appears unrelated to antibody titre. Besides, it may also suggest beneficial use of rituximab in the early phase of anti-MAG neuropathy.
Le texte complet de cet article est disponible en PDF.Mots clés : Rituximab, Monoclonal IgM gammopathy, Myelin-associated glycoprotein
Plan
Vol 174 - N° S1
P. S165 - avril 2018 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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