Neuropsychiatric lupus and hormones.

Neuropsychiatric lupus can present with a broad spectrum of severity, ranging from mild chronic symptomatology to acute life threatening illness. There are no accepted diagnostic or classification criteria, but most agree that the manifestations can be described as representing diffuse CNS insult or a more focal or localized process. The pathogenesis is unknown, but it is likely that there is more than one mechanism given the heterogeneous clinical presentations. It is probable that autoantibody formation, vascular injury, and cytokine production all play a role. There are no randomized controlled trials to help guide therapeutic decision making, but there is data to suggest that treatment of severe NPSLE with IV cyclophosphamide may be of benefit. Additionally, there may be a role for IVGG or synchronized plasmapheresis in cases that fail to respond to IV-CYC. An important role for sex steroids in the etiology of NPSLE is suggested by various observations. Investigational therapy with dehydroepiandrosterone (DHEA) appeared to have benefits with respect to milder symptoms of NPSLE.