Bouchikh M, Zouaidia F, Benhaddou E H A, Mahassini N, Achir A, El Malki H O
Thoracic Surgery Department, Ibn Sina University Hospital, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco; Research Center in Clinical Epidemiology and Clinical Trials (CRECET), Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco.
Anatomopathology Department, Ibn Sina University Hospital, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco.
Rev Neurol (Paris). 2017 Jun;173(6):388-395. doi: 10.1016/j.neurol.2017.03.020. Epub 2017 Apr 28.
The receptor for advanced glycation end-products (RAGE) is a membranous immunoglobulin involved in the pathogenesis of numerous autoimmune diseases and tumors. The aim of this study was to investigate the possible involvement of RAGE in the pathogenesis of myasthenia gravis.
This prospective study included 41 cases of myasthenia gravis treated at our institution between 2010 and 2015. There were 18 men and 23 women, with an average age of 36.44±14.47 years. The majority of patients (24.4%) were classified as IIb, according to MGFA scoring, and 21 of them required corticosteroid and/or immunosuppressive treatment. Assessment of RAGE in thymus specimens was done by immunohistochemistry using RAGE antibody (C-term). RAGE expression was assessed according to various clinical, paraclinical and pathological parameters.
Histopathological studies found 18 thymomas, 17 hyperplasias and six other types of pathology. Expression of RAGE was negative/weak in 19 cases and moderate/strong in 22 cases. It was more important in thymoma type B2 (P<0.001) and when the duration of myasthenia was short (P=0.04), and was not significantly related to either myasthenia clinical severity or preoperative treatment.
Our results suggest that the RAGE pathway is involved in myasthenia gravis pathophysiology, especially at disease onset, and in forms with thymomas. Further studies would be indispensable to explore other aspects of this signaling pathway, especially the potential role of different ligands and soluble forms of RAGE.
晚期糖基化终末产物受体（RAGE）是一种膜免疫球蛋白，参与多种自身免疫性疾病和肿瘤的发病机制。本研究旨在探讨RAGE在重症肌无力发病机制中的可能作用。
本前瞻性研究纳入了2010年至2015年在我院接受治疗的41例重症肌无力患者。其中男性18例，女性23例，平均年龄36.44±14.47岁。根据MGFA评分，大多数患者（24.4%）被归类为IIb型，其中21例需要糖皮质激素和/或免疫抑制治疗。使用RAGE抗体（C端）通过免疫组织化学法评估胸腺标本中的RAGE。根据各种临床、副临床和病理参数评估RAGE表达。
组织病理学研究发现18例胸腺瘤、17例增生和6例其他类型的病变。19例RAGE表达为阴性/弱阳性，22例为中度/强阳性。在B2型胸腺瘤中更明显（P<0.001），重症肌无力病程较短时也更明显（P=0.04），且与重症肌无力的临床严重程度或术前治疗均无显著相关性。
我们的结果表明，RAGE途径参与重症肌无力的病理生理过程，尤其是在疾病发作时以及胸腺瘤形式中。进一步研究探索该信号通路的其他方面不可或缺，特别是不同配体和RAGE可溶性形式的潜在作用。
