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This work is supported by Fondo de Investigaciones Sanitarias (FIS) grant FIS19/01774, Instituto de Salud Carlos III and cofunded by the European Union (ERDF/ESF, A Way to Make Europe/Investing in Your Future). Rodrigo Alvarez--Velasco was supported by a PhD for Medical Doctors grant from the Pla Estrategic de Recerca i Innovacio en Salut (PERIS), Generalitat de Catalunya (SLT008/18/00207). Elena Cortes-Vicente was supported by a Juan Rodes grant (JR19/00037) from the Fondo de Investigacion en Salud, Instituto de Salud Carlos III, Ministry of Health (Spain).

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Clinical characteristics and outcomes of thymoma-associated myasthenia gravis

Publicated to:European Journal Of Neurology. 28 (6): 2083-2091 - 2021-03-30 28(6), DOI: 10.1111/ene.14820

Authors: Alvarez-Velasco, Rodrigo; Gutierrez-Gutierrez, Gerardo; Carlos Trujillo, Juan; Martinez, Elisabeth; Segovia, Sonia; Arribas-Velasco, Marina; Fernandez, Guillermo; Paradas, Carmen; Velez-Gomez, Beatriz; Casasnovas, Carlos; Nedkova, Velina; Guerrero-Sola, Antonio; Ramos-Fransi, Alba; Martinez-Pineiro, Alicia; Pardo, Julio; Sevilla, Teresa; Gomez-Caravaca, Maria Teresa; Lopez de Munain, Adolfo; Jerico, Ivonne; Pelayo-Negro, Ana L; Asuncion Martin, Maria; Morgado, Yolanda; Dolores Mendoza, Maria; Perez-Perez, Helena; Rojas-Garcia, Ricard; Turon-Sans, Janina; Querol, Luis; Gallardo, Eduard; Illa, Isabel; Cortes-Vicente, Elena

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Abstract

Background and purpose Prognosis of myasthenia gravis (MG) in patients with thymoma is not well established. Moreover, it is not clear whether thymoma recurrence or unresectable lesions entail a worse prognosis of MG. 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.

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