Clinical characteristics and outcomes of thymoma‐associated myasthenia gravis

医学 胸腺瘤 重症肌无力 危险系数 置信区间 内科学 优势比 外科
作者
Rodrigo Álvarez‐Velasco,Gerardo Gutiérrez‐Gutiérrez,Juan Carlos Trujillo,Elisabeth Martínez,Sonia Segovia,Marina Arribas‐Velasco,Guillermo Fernández,Carmen Paradas,Beatriz Gómez,Carlos Casasnovas,Velina Nedkova,A. Guerrero-Solá,Alba Ramos‐Fransí,Alicia Martínez‐Piñeiro,Julio Pardo,Teresa Sevilla,María Teresa Gómez‐Caravaca,Adolfo López de Munaín,Ivonne Jericó,Ana L. Pelayo‐Negro
出处
期刊:European Journal of Neurology [Wiley]
卷期号:28 (6): 2083-2091 被引量:63
标识
DOI:10.1111/ene.14820
摘要

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.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.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.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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