Fig. 4
From: Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus (jSLE)
Proposed pathomechanisms of CNS vasculitis in NP-jSLE. Several, likely connected pathways may be involved in the pathogenesis of vasculitis in NP-jSLE. Circulating autoantibodies can recruit to vascular epithelia and/or form immune complexes that contribute to complement activation. Both, antibody binding and the presence of activated complement contribute to endothelial activation that, in turn, mediates the upregulation of adhesion molecules (including intercellular adhesion molecule 1/ICAM1 and vascular cell adhesion molecule 1/VCAM1) and the expression of pro-inflammatory cytokines, including IL-6 and IL-8. As a result, B and T lymphocytes are attracted, adhere to ICAM1/VCAM1 and infiltrate the perivascular region where they produce inflammatory cytokines and B cell survival factors (BAFF/BLys) [49]