Table 3 |.

Autoantibodies in NPSLE

Antibody	Effects in humans	Data from preclinical models	Refs
Anti-phospholipid antibodies: Anti-cardiolipin antibodies Anti-β2-glycoprotein 1 antibodies Lupus anticoagulant	Focal and diffuse disease due to medium-vessel and small-v essel vasculopathy Evidence of involvement in diffuse neuropsychiatric symptoms	Intracerebroventricular injection causes neurobehavioural deficits in mice	16,81–92
Anti-N-methyl-d-aspartate receptor antibodies	CSF positivity is correlated with NPSLE in some patients	Induce cognitive and affective symptoms in mice when introduced intrathecally	50,58–60,96–103
Anti-ribosomal P protein antibodies	Seropositivity has long been correlated with NPSLE	Bind to limbic system brain regions, thereby affecting mood and memory Induce TNF production in vitro, promoting inflammation and BBB impairment	30,54–56,105–115
Anti-aquaporin 4 antibodies	Associated with demyelination in neuromyelitis optica, found not only in NPSLE but also in SLE without neuropsychiatric features	ND	116–124
Anti-endothelial cell antibodies	Present in a majority of patients with NPSLE and in many with SLE	Might induce endothelial cell activation and expression of adhesion molecules or cytokines	125–128
Anti-MAP2 antibodies	100% correlated with CNS disease when found in the CSF Intracellular antigen, which suggests that neuron death might be required before they develop	MAP2 is exclusively found in neurons	129,130
Anti-suprabasin antibodies	Occur in high titres in patients with NPSLE Unclear mechanistic role in pathogenesis	Bind to an epidermal differentiation marker secreted by stratified epithelium In vitro, activate astrocyte senescence and autophagy	131–133

BBB, blood–brain barrier; CNS, central nervous system; CSF, cerebrospinal fluid; MAP2, microtubule-associated protein 2; ND, not discussed; NPSLE, neuropsychiatric systemic lupus erythematosus; SLE, systemic lupus erythematosus.