• Xiaojie Cao, Mingjie Zhang, Zhijie Lu, Congyang Li, Yanping Zeng, Jin Fan, and Ke Yu.
• 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): e25063e25063.

RationaleSystemic 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.Patient ConcernsIn 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.DiagnosesTaking into account the clinical manifestations and laboratory tests of the present case, the diagnosis of SLE and probable IIM was established.InterventionsCorticosteroids and additional gamma globulin were administered and the clinical symptoms were relieved during the treatment process.OutcomesUnfortunately, 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.LessonsTo 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.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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