Posterior reversible encephalopathy syndrome and autoimmunity

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome characterized by acute or subacute onset of neurological symptoms (e.g., headache, seizure, confusion, vomiting, and diminished eyesight) and impaired endothelial barrier function of the cerebral circulation that leads to bilateral subcortical vasogenic edema, while exhibiting a reversible feature in most cases. Clinically, various predisposing or precipitating conditions have been identified, such as hypertension, autoimmune diseases, renal dysfunction/failure, pre-eclampsia/eclampsia, post-transplantation conditions, and certain therapeutic agents. Among several putative mechanisms, the immune activation hypothesis prevails, as up to 50% of patients with PRES harbor abnor-malities related to autoimmunity, such as concurrent systemic lupus erythematosus. In this Review, we sum-marize the clinical and laboratory evidence that places PRES in the context of autoimmunity.

Automated summary

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome characterized by acute or subacute neurological symptoms and impaired endothelial barrier function, resulting in reversible bilateral subcortical vasogenic edema. Various predisposing or precipitating conditions, such as hypertension, autoimmune diseases, and certain therapeutic agents, have been identified. The immune activation hypothesis, especially the presence of autoimmunity, plays a significant role in PRES.