Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose:
Systemic lupus erythematosus (SLE) is caused by an imbalance of the immune system and may lead to injury and dysfunction of various systems and organs, including the central nervous system. Different attribution protocols have been developed in adult-onset SLE, however, none has been validated for childhood-onset SLE (cSLE). This study aimed to validate an attribution protocol for NP manifestations and to determine clinical significance of NP manifestations in cSLE.
Methods: A complete clinical, laboratory and neurological evaluation was performed in all subjects. Neurological manifestations were analyzed according to the ACR classification criteria. Cognitive evaluation was performed in all participants using Wechsler Intelligence Scale according to age and validated in Portuguese. Mood disorders were determined through Becks Depression and Becks Anxiety Inventory. Disease activity was measured by the Systemic Lupus Erythematosus Disease Activity Index and cumulative damage was determined using the Systemic Lupus International Collaborating Clinics Damage Index (SDI). Serum biomarkers (antiribossomal P anticardiolipin Anti-double stranded DNA, Ro (SSA), La (SSB), Sm, anticardiolipin lupus anticoagulant were evaluated.Dose of corticosteroids and other immunosuppressant medications were obtained by review of the medical charts. The protocol was developed and validated in adult-onset SLE by one of the authors (MG). In this protocol each NP manifestations is categorized in unrelated to SLE, uncertain relation to SLE and as definitively related to SLE according to disease duration, disease activity and antibody profile. According to our chart review some modifications were made to the original protocol, such as inclusion of macrophage activation syndrome.
Results:
A total of 161 NP were observed in 80 (73 female, mean age 19.13 SD± 4.57 years of 99 (80.8%) patients. Mean disease duration was 6.92(SD± 5.16) years. 39 (48.75%) of patients presented NPSLE during the first 6 months of disease. In decreasing order we observed the following NP manifestations: 68(85%) headache, 22(31.25%) cognitive impairment, 19 (23.75%) mood disorder, 16 (20%) seizure, 12 (15%) cerebrovascular disease, 12 (15%) anxiety, 3 (3.75%) acute confusional state, 3 (3.75%) psychosis, 2(2.5%) corea, 1 (1.25%) cranial neuropathy, 1 (1.25%) aseptic meningitis, 1 (1.25%) demyelinating syndrome and 1 (1.25%) polyneuropathy. Positive anticardiolipin antibodies were identified in 27 (27.27%), positive LA in 28 (28.28%) and positive anti-P in 9/34 (26.47%) cSLE patients. Using the attribution protocol NP manifestations were classified as unrelated to SLE in 1(1.25%) events, uncertain expressions to SLE in 47(58.75%) and as definitively related to SLE 32 (40.0%) events. The NPSLE were associated with azathioprine (p = 0.011), current age (p = 0.006), disease duration (p=0.023) and corticosteroid use (p 0.019).
Conclusion: Neuropsychiatric manifestations are prevalent in cSLE, however only 40% are definitively attributed to SLE. Results are similar to adult-onset SLE. This protocol has to be further validated in other cohorts, but may be useful in clinical practice.
Disclosure:
R. Barbosa,
None;
K. O. Peliçari,
None;
A. T. Lapa,
None;
N. A. Sinicato,
None;
M. Postal,
None;
R. Marini,
None;
M. Govoni Sr.,
None;
S. Appenzeller,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/attribution-protocol-and-clinical-significance-of-neuropsychiatric-manifestations-in-childhood-onset-systemic-lupus-erythematosus/