Clinical and therapeutic features of myasthenia gravis in adults based on age at onset

Elena Cortés-Vicente, Rodrigo Álvarez-Velasco, Sonia Segovia, Carmen Paradas, Carlos Casasnovas, Antonio Guerrero-Sola, Julio Pardo, Alba Ramos-Fransi, Teresa Sevilla, Adolfo López De Munain, Maria Teresa Gómez, Ivonne Jericó, Gerardo Gutiérrez-Gutiérrez, Ana Lara Pelayo-Negro, María Asunción Martín, María Dolores Mendoza, Germán Morís, Ricard Rojas-Garcia, Jordi Díaz-Manera, Luis QuerolEduard Gallardo, Beatriz Vélez, María Antonia Albertí, Lucía Galán, Tania García-Sobrino, Alicia Martínez-Piñeiro, Ana Lozano-Veintimilla, Roberto Fernández-Torrón, Ángel Cano-Abascal, Isabel Illa*

*Autor corresponent d’aquest treball

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ObjectiveTo describe the characteristics of patients with very-late-onset myasthenia gravis (MG).MethodsThis observational cross-sectional multicenter study was based on information in the neurologist-driven Spanish Registry of Neuromuscular Diseases (NMD-ES). All patients were >18 years of age at onset of MG and onset occurred between 2000 and 2016 in all cases. Patients were classified into 3 age subgroups: early-onset MG (age at onset <50 years), late-onset MG (onset =50 and <65 years), and very-late-onset MG (onset =65 years). Demographic, immunologic, clinical, and therapeutic data were reviewed.ResultsA total of 939 patients from 15 hospitals were included: 288 (30.7%) had early-onset MG, 227 (24.2%) late-onset MG, and 424 (45.2%) very-late-onset MG. The mean follow-up was 9.1 years (SD 4.3). Patients with late onset and very late onset were more frequently men (p < 0.0001). Compared to the early-onset and late-onset groups, in the very-late-onset group, the presence of anti-acetylcholine receptor (anti-AChR) antibodies (p < 0.0001) was higher and fewer patients had thymoma (p < 0.0001). Late-onset MG and very-late-onset MG groups more frequently had ocular MG, both at onset (<0.0001) and at maximal worsening (p = 0.001). Although the very-late-onset group presented more life-threatening events (Myasthenia Gravis Foundation of America IVB and V) at onset (p = 0.002), they required fewer drugs (p < 0.0001) and were less frequently drug-refractory (p < 0.0001).ConclusionsPatients with MG are primarily =65 years of age with anti-AChR antibodies and no thymoma. Although patients with very-late-onset MG may present life-threatening events at onset, they achieve a good outcome with fewer immunosuppressants when diagnosed and treated properly.

Idioma originalAnglès
Pàgines (de-a)e1171-e1180
RevistaNeurology
Volum94
Número11
DOIs
Estat de la publicacióPublicada - 17 de març 2020

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