Clinical and therapeutic features of myasthenia gravis in adults based on age at onset
Por:
Cortes-Vicente, E, Alvarez-Velasco, R, Segovia, S, Paradas, C, Casasnovas, C, Guerrero-Sola, A, Pardo, J, Ramos-Fransi, A, Sevilla, T, de Munain, AL, Gomez, MT, Jerico, I, Gutierrez-Gutierrez, G, Pelayo-Negro, AL, Martin, MA, Mendoza, MD, Moris, G, Rojas-Garcia, R, Diaz-Manera, J, Querol, L, Gallardo, E, Velez, B, Alberti, MA, Galan, L, Garcia-Sobrino, T, Martinez-Pineiro, A, Lozano-Veintimilla, A, Fernandez-Torron, R, Cano-Abascal, A and Illa, I
Publicada:
17 mar 2020
Ahead of Print:
18 feb 2020
Resumen:
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.
Filiaciones:
Cortes-Vicente, E:
Hosp Santa Creu & Sant Pau, Dept Neurol, Neuromuscular Dis Unit, Barcelona, Spain
Univ Autonoma Barcelona, Dept ofMedicine, Barcelona, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Alvarez-Velasco, R:
Hosp Santa Creu & Sant Pau, Dept Neurol, Neuromuscular Dis Unit, Barcelona, Spain
Univ Autonoma Barcelona, Dept ofMedicine, Barcelona, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Segovia, S:
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Paradas, C:
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Neurodegenerat, Madrid, Spain
Univ Seville, Inst Biomed Sevilla, Hosp U Virgen Rocio, Neurol Dept,Neuromuscular Disorders Unit,CSIC, Seville, Spain
Casasnovas, C:
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Hosp Univ Bellvitge IDIBELL, Hosp Llobregat, Neurol Dept, Unitat Neuromuscular, Barcelona, Spain
Guerrero-Sola, A:
Hosp Univ Clin San Carlos, Inst Neurosci, Dept Neurol, Neuromuscular Dis Unit, Madrid, Spain
Pardo, J:
Hosp Clin, Neurol Dept, Santiago De Compostela, Spain
:
Hosp Badalona Germans Trias & Pujol, Dept Neurol, Neuromuscular Dis Unit, Badalona, Spain
Univ Autonoma Barcelona, Dept Med, Badalona, Spain
Sevilla, T:
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Hosp Univ & Politecn La Fe, Neurol Dept, Neuromuscular Unit, Valencia, Spain
Univ Valencia, Dept Med, Valencia, Spain
de Munain, AL:
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Neurodegenerat, Madrid, Spain
Hosp Univ & Politecn La Fe, Neurol Dept, Neuromuscular Unit, Valencia, Spain
Donostia Univ Hosp, Neurol Dept, San Sebastian, Spain
Univ Basque Country, Biodonostia Res Inst, Neurosci Area, San Sebastian, Spain
Gomez, MT:
Hosp Univ Reina Sofia, Neurol Dept, Cordoba, Spain
Jerico, I:
Complejo Hosp Navarra, Dept Neurol, Pamplona, Spain
Gutierrez-Gutierrez, G:
Univ Europea Madrid, Hosp Univ Infanta Sofia, Dept Neurol, Neuromuscular Dis Unit, Madrid, Spain
Pelayo-Negro, AL:
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Neurodegenerat, Madrid, Spain
Univ Cantabria, Univ Hosp Marques Valdecilla IDIVAL, Serv Neurol, Santander, Spain
Martin, MA:
Complejo Asistencial Hosp Burgos, Burgos, Spain
Mendoza, MD:
Hosp Univ Gran Canaria Doctor Negrin, Las Palmas Gran Canaria, Spain
Moris, G:
Hosp Cent Asturias, Oviedo, Spain
Rojas-Garcia, R:
Hosp Santa Creu & Sant Pau, Dept Neurol, Neuromuscular Dis Unit, Barcelona, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Diaz-Manera, J:
Hosp Santa Creu & Sant Pau, Dept Neurol, Neuromuscular Dis Unit, Barcelona, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Querol, L:
Hosp Santa Creu & Sant Pau, Dept Neurol, Neuromuscular Dis Unit, Barcelona, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Gallardo, E:
Hosp Santa Creu & Sant Pau, Dept Neurol, Neuromuscular Dis Unit, Barcelona, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Velez, B:
Univ Seville, Inst Biomed Sevilla, Hosp U Virgen Rocio, Neurol Dept,Neuromuscular Disorders Unit,CSIC, Seville, Spain
Alberti, MA:
Hosp Univ Bellvitge IDIBELL, Hosp Llobregat, Neurol Dept, Unitat Neuromuscular, Barcelona, Spain
Galan, L:
Hosp Univ Clin San Carlos, Inst Neurosci, Dept Neurol, Neuromuscular Dis Unit, Madrid, Spain
Garcia-Sobrino, T:
Hosp Clin, Neurol Dept, Santiago De Compostela, Spain
:
Hosp Badalona Germans Trias & Pujol, Dept Neurol, Neuromuscular Dis Unit, Badalona, Spain
Lozano-Veintimilla, A:
Hosp Univ & Politecn La Fe, Neurol Dept, Neuromuscular Unit, Valencia, Spain
Fernandez-Torron, R:
Donostia Univ Hosp, Neurol Dept, San Sebastian, Spain
Cano-Abascal, A:
Univ Cantabria, Univ Hosp Marques Valdecilla IDIVAL, Serv Neurol, Santander, Spain
Illa, I:
Hosp Santa Creu & Sant Pau, Dept Neurol, Neuromuscular Dis Unit, Barcelona, Spain
Univ Autonoma Barcelona, Dept ofMedicine, Barcelona, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
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