A followup study of antiphospholipid antibodies and associated neuropsychiatric manifestations in 137 children with systemic lupus erythematosus

Abstract

Objective. To determine the prevalence of anticardiolipin antibodies (aCL), anti-␤ 2 -glycoprotein I (anti-␤ 2 GPI) antibodies, and lupus anticoagulant (LAC) in a large cohort of children with systemic lupus erythematosus (SLE), and to evaluate the associations with neuropsychiatric manifestations. Methods. A single-center retrospective cohort study with longitudinal followup of antiphospholipid antibodies (aPL) in 137 children with SLE (25 boys and 112 girls, mean age at diagnosis 13.0 years) was performed. Patients were followed up for a mean of 31 months. Results. At the time of diagnosis, 65% of the children were aCL positive, 41% had anti-␤ 2 GPI antibodies, and 26% were LAC positive. Analysis of the association between presence of aPL and individual neuropsychiatric manifestations at diagnosis showed a statistically significant association of positive LAC with cerebrovascular disease (5 patients; P ‫؍‬ 0.015). A persistently positive aCL was observed in 50%, anti-␤ 2 GPI antibodies in 29%, and LAC in 16% of children over time. The prevalence of anti-␤ 2 GPI antibodies, but not aCL and LAC, was found to be statistically significantly higher in children with neuropsychiatric disease compared with those without (P ‫؍‬ 0.02). Comparison for specific neuropsychiatric manifestations showed a statistically significant association between a persistently positive LAC and chorea (2 patients; P ‫؍‬ 0.02). Conclusion. The prevalence of anti-␤ 2 GPI antibodies was found to be higher in the group of SLE patients with neuropsychiatric disease compared with those without. Our data suggest an association between LAC and cerebrovascular disease at the time of SLE diagnosis and chorea over the disease course, but not between aPL and other neuropsychiatric manifestations.

Generalized chorea as the first presentation of systemic lupus erythematosus: case report and review of literature

“…However, NLSLE pathogenesis is still controversial, and probably involve immune mediated as well as ischemic mechanisms. 16,17 In our patient, the old and new ischemic changes noted on MRI are consistent with an ischemic vascular process, but these can't be explained simply by the procoagulant effects of APL antibodies causing thrombotic occlusion of capillaries. It was hypothesized that an immune process may be responsible for a direct injury to the blood-brain barrier allowing autoantibodies to enter the nervous system and attack neurons involved in nigro-striatal pathways.…”

“…It was hypothesized that an immune process may be responsible for a direct injury to the blood-brain barrier allowing autoantibodies to enter the nervous system and attack neurons involved in nigro-striatal pathways. [16][17][18][19] One meta-analysis showed that NPSLE patients have higher serum levels of certain antibodies including anticardiolipin, lupus anticoagulant, anti-ribosomal P, and antineuronal antibodies when compared with SLE patients who have no neurological involvement. 20 Other secondary factors that also contribute to the pathogenesis include infections associated with immunosuppressive treatments, hypertension, and renal failure.…”

Associação entre doenças imunológicas e suas manifestações clínicas semelhantes

“…Lupic chorea is uncommon, being observed in 2% of cases of SLE 3 . As in rheumatic fever, it occurs in children aged around 11 years, especially females and it may be indistinguishable from Sydenham's chorea 3,5 .…”

“…Different studies have reported APS as the primary cause of chorea 3,5,8 . It is rare manifestation and occurs especially in females.…”

Acute inflammatory myelopathies

“…In a study of 137 children with neuropsychiatric SLE, two children had transverse myelitis. Both tested positive for anticardiolipin antibodies and anti-b 2 glycosylphosphatidylinositol antibodies (1 was positive for lupus anticoagulant) (Avcin et al, 2008).…”