Clinical characteristics and outcomes of thymoma-associated myasthenia gravis.

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.

• Authors: Rodrigo Álvarez-Velasco, Gerardo Gutiérrez-Gutiérrez, Juan Carlos Trujillo, Elisabeth Martínez, Sonia Segovia, Marina Arribas-Velasco, Guillermo Fernández, Carmen Paradas, Beatriz Vélez-Gómez, Carlos Casasnovas, Velina Nedkova, Antonio Guerrero-Sola, Alba Ramos-Fransi, Alicia Martínez-Piñeiro, Julio Pardo, Teresa Sevilla, María Teresa Gómez-Caravaca, Adolfo López de Munain, Ivonne Jericó, Ana L Pelayo-Negro, María Asunción Martín, Yolanda Morgado, María Dolores Mendoza, Helena Pérez-Pérez, Ricard Rojas-García, Janina Turon-Sans, Luis Querol, Eduard Gallardo, Isabel Illa, Elena Cortés-Vicente
• Journal: European journal of neurology (Eur J Neurol) Vol. 28 Issue 6 Pg. 2083-2091 (06 2021) ISSN: 1468-1331 [Electronic] England
• PMID: 33721382 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: © 2021 European Academy of Neurology.

Topics

• Humans
• Myasthenia Gravis (complications, epidemiology)
• Neoplasm Recurrence, Local
• Retrospective Studies
• Thymectomy
• Thymoma (complications, epidemiology)
• Thymus Neoplasms (complications, epidemiology)