Department of Internal Medicine and Clinical Immunology, Université Paris-Saclay, AP-HP, Le Kremlin-Bicêtre, France.
Department of Internal Medicine and Clinical Immunology, Université Paris-Saclay, AP-HP, Le Kremlin-Bicêtre, France; Inserm, CEA, Université Paris Saclay, centre de recherche en immunologie des infections virales et des maladies auto-immunes ImVA, UMR1184, 94270 Le Kremlin-Bicêtre, France.
Rev Med Interne. 2021 Aug;42(8):575-578. doi: 10.1016/j.revmed.2021.05.006. Epub 2021 May 26.
Onconeuronal antibodies directed against intracellular antigens are strongly associated with paraneoplastic syndromes and their detection in the absence of cancer is unusual. We herein report a case of anti-Ma2 encephalitis associated with Sjogren's syndrome (SS).
An 81-year-old woman followed for a cutaneous lupus with vasculitis associated with SS presented a flare of her disease with neurological worsening including walking difficulty, hypersialorrhea and dysphagia. A paraneoplastic origin of the symptoms was suspected and anti-Ma2 antibodies were positive in serum. The search for an underlying neoplasia was negative. The diagnosis of anti-Ma2 encephalitis secondary to a SS was made. In the literature, the association of anti-Ma2 encephalitis and SS has been previously reported twice. Cases of patients with other onconeuronal antibodies associated with SS have been also reported. Anti-Ma2 encephalitis is a rare condition with a wide spectrum of symptoms associated with a cancer in more than 90% of the cases. Anti-Ma2 encephalitis has also been described after the use of immune check points inhibitors underscoring the role of autoimmunity in its pathogenesis.
Anti-Ma2 encephalitis is essentially associated with neoplasia but can occur in Sjogren's syndrome.
针对细胞内抗原的癌相关抗体与副肿瘤综合征强烈相关，在没有癌症的情况下检测到这些抗体是不寻常的。本文报告了一例与干燥综合征（SS）相关的抗 Ma2 脑炎。
一名 81 岁女性因皮肤狼疮伴血管炎相关 SS 而接受治疗，她的疾病出现了复发，伴有神经恶化，包括行走困难、唾液分泌过多和吞咽困难。怀疑症状是副肿瘤性的，血清中存在抗 Ma2 抗体。未发现潜在的肿瘤。诊断为继发于 SS 的抗 Ma2 脑炎。在文献中，抗 Ma2 脑炎和 SS 的关联此前曾被报道过两次。还报道了与 SS 相关的其他癌相关抗体的患者病例。抗 Ma2 脑炎是一种罕见的疾病，其症状广泛，与 90%以上的癌症相关。免疫检查点抑制剂的使用后也描述了抗 Ma2 脑炎，这突出了自身免疫在其发病机制中的作用。
抗 Ma2 脑炎主要与肿瘤有关，但也可发生在干燥综合征中。
