Background The neuropsychiatric (NP) manifestations are an important cause of morbidity and mortality in patients with pediatric-onset systemic lupus erythematosus (pSLE). The prevalence of these manifestations ranges from 29% to 44% in different series, but data in the spanish pediatric population are scarce.
Objectives To analyze the clinical and immunological features of patients with pSLE and NP followed in a Spanish tertiary center.
Methods We performed a retrospective study of 49 patients with pSLE diagnosed between 1985 and 2005 in our center. The ACR NPSLE case definitions were used for classification. Demographic, clinical and immunological data were obtained through review of their medical charts.
Results Twenty six patients (53%) developed NP manifestations. Mean age was 13.6 years and female:male ratio was 2.7:1. NP manifestations were present at the beginning of the disease in 7 cases (26.9%) and the mean time to develop them was 30.5 months. The most common presentations of NPSLE were seizures (50%), headache (26.9%), mood disorder/depression (26.9%), psychosis (19.2%), cerebrovascular disease (15.3%) and aseptic meningitis (15.3%). There was more than one NP manifestation in 15 cases (57.7%), with an average of 2.4 manifestations/patient. The comparison of patients with and without NPSLE demonstrated significant diffences (p<0.05) in the number of males, titers of antinuclear antibodies (ANA) and anti-DNA antibodies, positivity for anti-β2-glicoprotein I (β2GPI) and cryoglobulins, high erythrosedimentation rate (ESR) and low complement (C3, C4) (Table 1). There were 2 cases of mortality in NPSLE (7.7%) during follow-up period, one as a result of infection of the central nervous system and another due to sepsis associated with intestinal thrombosis.
Conclusions In our series >50% of patients had NPSLE manifestations and frequently occurred early during the course of the disease. The clinical spectrum of NPSLE was wide in our cases and most of them had more than one manifestation. Patients with NPSLE showed a higher disease activity as measured by levels of autoantibodies, increased acute-phase reactants and low complement. Although NPSLE has been associated with antiphospholipid antibodies in other series, specifically anticardiolipin antibodies and lupus anticoagulant, we only found significant association with anti-β2GPI antibodies. Mortality in patients with NPSLE was high.
Disclosure of Interest None declared