Multiple neurological manifestations in a patient with systemic lupus erythematosus and anti-NXP2-positive myositis

Abstract

Rationale: 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. Patient concerns: 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. Diagnoses: Taking into account the clinical manifestations and laboratory tests of the present case, the diagnosis of SLE and probable IIM was established. Interventions: Corticosteroids and additional gamma globulin were administered and the clinical symptoms were relieved during the treatment process. Outcomes: 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. Lessons: 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.

Profiling of Myositis Specific Antibodies and Composite Scores as an Aid in the Differential Diagnosis of Autoimmune Myopathies

“…Whether the reactivity in the ID patient represents a pathogenic mechanism as discussed previously deserved further research [ 41 , 42 ]. Besides in IIM, anti-NXP-2 antibodies have been described in a patient with SLE and neurological complications [ 43 ].…”

Clinical value of chemiluminescence method for detection of antinuclear antibody profiles

“…On the other hand, CLIA utilizes a homogeneous liquid-phase reaction system that offers improved reaction efficiency and a more comprehensive cleaning step. This can lead to a significant enhancement in the specificity of the reaction system[ 22 ]. Moreover, the diagnostic performance of these antibodies detected by both methods was found to be similar for SLE, and ROC analysis of the diagnostic model consisting of anti-dsDNA antibody and anti-Ro60 antibody detected by CLIA exhibited that the AUC values, sensitivity, and specificity of the model were 0.997, 0.962, and 0.978.…”