Clinical characteristics and outcomes of thymoma-associated myasthenia gravis.
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Álvarez-Velasco Rodrigo, Gutiérrez-Gutiérrez Gerardo, Trujillo Juan Carlos, Martínez Elisabeth, Segovia Sonia, Arribas-Velasco Marina, Fernández Guillermo, Paradas Carmen, Vélez-Gómez Beatriz, Casasnovas Carlos, Nedkova Velina, Guerrero-Sola Antonio, Ramos-Fransi Alba, Martínez-Piñeiro Alicia, Pardo Julio, Sevilla Teresa, Gómez-Caravaca María Teresa, López de Munain Adolfo, Jericó Ivonne, Pelayo-Negro Ana L, Martín María Asunción, Morgado Yolanda, Mendoza María Dolores, Pérez-Pérez Helena, Rojas-García Ricard, Turon-Sans Janina, Querol Luis, Gallardo Eduard, Illa Isabel, Cortés-Vicente Elena
Аннотация
METHODS: This multicenter study was based on data from a Spanish neurologist-driven MG registry. All patients were aged >18 years at onset and had anti-acetylcholine receptor antibodies. We compared the clinical data of thymomatous and nonthymomatous patients. Prognosis of patients with recurrent or nonresectable thymomas was assessed.
RESULTS: We included 964 patients from 15 hospitals; 148 (15.4%) had thymoma-associated MG. Median follow-up time was 4.6 years. At onset, thymoma-associated MG patients were younger (52.0 vs. 60.4 years, p < 0.001), had more generalized symptoms (odds ratio [OR]: 3.02, 95% confidence interval [CI]: 1.95-4.68, p < 0.001) and more severe clinical forms according to the Myasthenia Gravis Foundation of America (MGFA) scale (OR: 1.6, 95% CI: 1.15-2.21, p = 0.005). Disease severity based on MGFA postintervention status (MGFA-PIS) was higher in thymomatous patients at 1 year, 5 years, and the end of follow-up. Treatment refractoriness and mortality were also higher (OR: 2.28, 95% CI: 1.43-3.63, p = 0.001; hazard ratio: 2.46, 95% CI: 1.47-4.14, p = 0.001). Myasthenic symptoms worsened in 13 of 27 patients with recurrences, but differences in long-term severity were not significant. Fifteen thymomatous patients had nonresectable thymomas with worse MGFA-PIS and higher mortality at the end of follow-up.
CONCLUSIONS: Thymoma-associated MG patients had more severe myasthenic symptoms and worse prognosis. Thymoma recurrence was frequently associated with transient worsening of MG, but long-term prognosis did not differ from nonrecurrent thymoma. Patients with nonresectable thymoma tended to present severe forms of MG.