Background/Purpose: Neuropsychiatric (NP) manifestations are a main cause of morbidity and mortality in juvenile-onset systemic lupus erythematosus (jSLE). Some studies suggest that they are more frequent and severe in jSLE than in adult-onset SLE (aSLE). We performed a study to compare the clinical and serological profile of pediatric and adult patients with neuropsychiatric lupus (NPSLE) treated in a Spanish tertiary center.

Methods: A retrospective study of patients with jSLE (age of onset: 0-18y) and aSLE (age of onset: >18y) seen in our center during the period 1988-2016 was performed. Case definitions of the American College of Rheumatology were used to identify NPSLE manifestations. Demographics, clinical and serological data were obtained through a review of their medical records.

Results: A total of 69 patients with NPSLE were included, aSLE 41 (59%) and jSLE 28 (41%), the comparison of groups is presented in the table. Most of them were Caucasian (92%), mean age at diagnosis in adults was 36.4 years (range: 19-68) and 13.9 years (range: 8-18) in children. The proportion of males was higher in the latter group. The mean duration of the disease was significantly greater in adults, as well as the time from SLE diagnosis to NP manifestation onset, although without significant difference. Central NP manifestations were the most frequent in both groups (aSLE 93%, jSLE 96%) regarding to the peripheral manifestations (aSLE 12%, jSLE 11%). The most frequent manifestations in aSLE were headache (29%), cerebrovascular disease (27%), seizures (17%) and myelopathy (15%), whereas in jSLE were seizures (46%), headache (29%), mood disorder/depression (25%), psychosis (18%) and autonomic disorders (18%). A significant group of patients presented ≥ 2 central manifestation during their evolution (aSLE 32%, jSLE 41%), with the mean number of manifestations in adults being 1.36 (range: 1-3) and in children 1.44 Range: 1-4 ). Patients with jSLE developed lupus nephritis (LN) with a significantly higher frequency, as well as higher titres of anti-DNA antibodies, erythrocyte sedimentation rate (ESR) and hypocomplementemia. During the study period there was mortality in 2 cases of aSLE and 2 jSLE (5% and 7%, respectively).

Conclusion: Our results corroborate that juvenile patients with NPSLE present higher disease activity compared to adults. There was no significant diference in the time from SLE diagnosis to NP manifestation onset, but tended to be shorter in jSLE. The spectrum of NPSLE was varied both groups and an important proportion developed ≥ 2 manifestation. Mortality continues to be important in NPSLE in both age groups.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparison-of-clinical-and-serological-features-of-juvenile-and-adult-onset-neuropsychiatric-lupus-in-spanish-patients/