No abstract

Computerized batteries have been widely used to investigate cognitive impairment (CI) in patients with SLE. The aim of this study was to evaluate the cognitive performance of patients with SLE in relation to healthy controls using the Pediatric Automated Neuropsychological Assessment Metrics (Ped-ANAM) battery. In addition, we aimed to examine differences in Ped-ANAM scores according to age of disease onset, presence of disease activity, and disease damage. We included 201 consecutive adult-onset (aSLE) and childhood-onset SLE (cSLE) patients who were being followed at the hospital’s rheumatology outpatient clinic and 177 healthy controls. We applied the percentage of correct answers on the Ped-ANAM subtests and the Performance Validity Index (PVI) metric and correlated them with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus Erythematosus Damage Index (SDI). Then, we established their relationships with neuropsychiatric systemic lupus erythematosus (NPSLE). We observed CI in a total of 38 (18.9%) SLE patients and 8 (4.5%) healthy controls (p < 0.001). CI was observed in eight (19.5%) cSLE patients and 32 (20%) aSLE patients (p = 0.8175). Individual analysis of the aSLE subtests showed a significant difference in all subtests compared to healthy controls; the greatest differences were in matching to sample (p < 0.001) and memory search ( p < 0.001). In the cSLE group, we observed a difference in the code substitution subtests (p = 0.0065) compared to the healthy controls. In the evaluation of clinical outcomes, disease activity was significantly correlated with CI in cSLE (r = 0.33; p = 0.042) and aSLE (r = 0.40; p = 0.001). We also observed an association between disease activity and neuropsychiatric manifestations (p = 0.0012) in aSLE. In conclusion, we determined that cognitive dysfunction, mainly in memory and attention, was more prevalent in patients with SLE. In both the cSLE and aSLE groups, disease activity was associated with worse cognitive function. This is the first study to use the Ped-ANAM in Brazil. Longitudinal studies are necessary to determine how the Ped-ANAM will perform over time.

Peripheral neuropathy (PN) is frequently observed in systemic rheumatic diseases and is a challenge in clinical practice. We aimed to review the evidence on the subject and proposed a comprehensive approach to these patients, facilitating diagnosis and management. We searched the MEDLINE database for the terms (and its respective Medical Subject Headings (MeSH) terms): “peripheral neuropathy” AND “rheumatic diseases” OR “systemic lupus erythematosus”, “rheumatoid arthritis”, “Sjogren syndrome”, and “vasculitis” from 2000 to 2023. This literature review focuses on the diagnostic workup of PNs related to systemic lupus erythematosus, Sjögren’s syndrome, rheumatoid arthritis, and systemic vasculitis. For every type of PN, we provide a pragmatic flowchart for diagnosis and also describe evidence-based strategies of treatment.

BACKGROUNDAnti-NMDAR autoimmune encephalitis (AIE) is a disease mediated mainly by NR1 autoantibodies against surface protein epitopes causing reversible neuronal damage. Systemic lupus erythematosus (SLE) is a chronic and multisystemic autoimmune disease that can significantly affect the central nervous system (CNS), mediated primary by NR2 antibodies. We will report a case of a patient with anti-NMDAR autoimmune encephalitis that developed SLE during follow-up period. CASE REPORTA 22-year-old female patient admitted with seizures, dysarthria and chorea. CSF analysis showed increased proteins, and absence of infections. Autoantibody screening revealed anti-NMDA receptor antibody t. With these results, the diagnosis of autoimmune encephalitis was established. Treatment with azathioprine was started, after the end of treatment, the patient evolved with complete clinical remission. One year after the initial treatment, the patient returns to the service with arthritis, malar rash and lymph node enlargement. Serum autoantibody screening revealed antinuclear antibody reagent with coarse speckled nuclear pattern (titer 1/1280); anti-dsDNA positivity and complement consumption. Treatment with methotrexate, prednisone, hydroxychloroquine was started, with complete remission. No CNS additional CNS involvement was noted. CONCLUSIONSystemic lupus erythematosus can mimic or be associated with EAI; however, autoantibodies profiles are often distinct. Initially, our patient had only AIE, with no evidence of SLE disease activity. Although the family of anti-glutamate receptor can be found in both diseases, we generally observed distinct subtypes. While NR1 is observed in AIE, NR2 is associated with CNS involvement in SLE. In summary, further studies are needed to follow-up AIE patients to determine the frequency of this association.