Department of Neurology, The General Hospital of Western Theater Command, Jinniu District, Chengdu, Sichuan Province, People's Republic of China.
Medicine (Baltimore). 2021 Mar 12;100(10):e25063. doi: 10.1097/MD.0000000000025063.
Systemic lupus erythematosus (SLE) is a complex autoimmune inflammatory disease that frequently affects various organs. Neuropsychiatric manifestations in SLE patients, known as neuropsychiatric SLE, are clinically common. However, the principal manifestation of cranial neuropathy in patients with SLE and comorbidities is relatively rare.
In this report, we describe a 51-year-old Chinese woman who was admitted with a chief complaint of chronic-onset facial paresthesia, dysphagia, and choking cough when drinking water, accompanied by slurred speech, salivation, and limb weakness. The blood autoantibody test results showed that many SLE-associated antibodies were positive. Meanwhile, anti-nuclear matrix protein 2 (NXP2) antibody was strongly positive in the idiopathic inflammatory myopathy (IIM) spectrum test from the serum. Muscle biopsy indicated inflammatory infiltration of the muscle fiber stroma.
Taking into account the clinical manifestations and laboratory tests of the present case, the diagnosis of SLE and probable IIM was established.
Corticosteroids and additional gamma globulin were administered and the clinical symptoms were relieved during the treatment process.
Unfortunately, the patient experienced sudden cardiac and respiratory arrest. Multiple system dysfunctions exacerbated disease progression, but in the present case, we speculated that myocardial damage resulting from SLE could explain why she suddenly died.
To our knowledge, multiple neurological manifestations in patients with SLE and anti-NXP2-positive myositis are rare. Note that SLE is still a life-threatening disease that causes multiple system dysfunctions, which requires increasing attention.
系统性红斑狼疮(SLE)是一种常见的自身免疫性炎症性疾病,常累及多个器官。SLE 患者的神经精神表现,即神经精神性 SLE,临床上较为常见。然而,SLE 合并其他疾病患者颅神经病的主要表现则相对少见。
本研究报告了一位 51 岁的中国女性患者,主要症状为慢性起病的面部感觉异常、吞咽困难和饮水呛咳,同时伴有言语含糊、流涎和四肢无力。血液自身抗体检测结果显示多种与 SLE 相关的抗体阳性。同时,血清特发性炎性肌病(IIM)谱检测中抗核基质蛋白 2(NXP2)抗体强阳性。肌肉活检提示肌纤维间质炎症浸润。
结合本病例的临床表现和实验室检查,诊断为 SLE 和可能的 IIM。
给予患者皮质类固醇和免疫球蛋白治疗,治疗过程中临床症状缓解。
遗憾的是,患者突发心脏和呼吸骤停。多器官功能障碍使疾病恶化,但在本病例中,我们推测 SLE 导致的心肌损伤可以解释其突然死亡的原因。
据我们所知,SLE 合并抗 NXP2 阳性肌炎患者出现多种神经表现较为罕见。需要注意的是,SLE 仍然是一种危及生命的疾病,可导致多器官功能障碍,应引起更多关注。