Available online 25 August 2022, 102892

The pathogenesis of lupus-specific autoantibodies distinguish NPLE from other CNS diseases.

Autoantibodies in the CSF of NPLE may raise from the meninges and choroid plexus.

Microglia are part of the first line of defense against antibodies-mediated damage.

Patients with systemic lupus erythematosus (SLE) frequently suffer from nervous system complications, termed neuropsychiatric lupus erythematosus (NPLE). NPLE accounts for the poor prognosis of SLE. Correct attribution of NP events to SLE is the primary principle in managing NPLE. The vascular injuries and neuroinflammation are the fundamental neuropathologic changes in NPLE. Specific autoantibody-mediated central nerve system (CNS) damages distinguish NPLE from other CNS disorders. Though the central antibodies in NPLE are generally thought to be raised from the periphery immune system, they may be produced in the meninges and choroid plexus. On this basis, abnormal activation of microglia and disease-associated microglia (DAM) should be the common mechanisms of NPLE and other CNS disturbances. Improved understanding of both characteristic and sharing features of NPLE might yield further options for managing this disease.

Neuropsychiatric lupus erythematosus

Autoantibodies

Microglia

Meninges

Choroid plexus

© 2022 Elsevier Ltd. All rights reserved.