Systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome and intracranial vasculopathy

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic condition characterized by reversible vasogenic edema on neuroimaging. It is associated with various neurological manifestations, including headaches, vomiting, seizures, visual loss, altered mental status and focal neurological deficits. PRES mainly occurs in the setting of eclampsia, hypertension, uremia, malignancy, transplantation, autoimmune diseases and/or use of immunosuppressive drugs. This syndrome has been described in patients with systemic lupus erythematosus (SLE). PRES is a potentially reversible clinical-radiological entity; however, it can be complicated with vasculopathy, infarction or hemorrhage. Vasculopathy has been demonstrated to be a common finding in patients with SLE. We report the case of a woman with lupus nephritis and PRES whose diffuse vasculopathy was present on initial neuroimaging. Subsequent brain computed tomography scan demonstrated interval development of intraparenchymal hemorrhage and subarachnoid hemorrhage. To our knowledge, this unique brain image pattern has not been reported in SLE patients.

Atypical Presentation of CLIPPERS Syndrome

“…11 On MRI, neuropsychiatric SLE can also demonstrate cytotoxic edema in acute stage. 12 Cerebrospinal fluid findings are similar to those of neuropsychiatric SLE. 12 Posterior reversible encephalopathy syndrome can be seen in the setting of eclampsia, malignant hypertension, uremia, transplantation, malignancy, autoimmune disorders (SLE), and/or immunosuppressive drugs (such as tacrolimus).…”

“…10 In 25% to 80% of the patients with neuropsychiatric SLE, oligoclonal IgG bands (OCBs) are detected, and treatment with corticosteroids leads to disappearance of the OCB. 12 Posterior reversible encephalopathy syndrome is characterized by reversible vasogenic edema on imaging, and it can be complicated by infarction, hemorrhage, and vascuolopathy. 11 Magnetic resonance imaging findings are variable and can demonstrate normal scan or cerebral atrophy, calcification, infarct intracranial hemorrhage, and/or subdural fluid collection.…”

“…Abnormally high or low levels of glucose and lactate are not usually present. 12 Posterior reversible encephalopathy syndrome can be seen in the setting of eclampsia, malignant hypertension, uremia, transplantation, malignancy, autoimmune disorders (SLE), and/or immunosuppressive drugs (such as tacrolimus). 11 On MRI, neuropsychiatric SLE can also demonstrate cytotoxic edema in acute stage.…”

“…11 Magnetic resonance imaging findings are variable and can demonstrate normal scan or cerebral atrophy, calcification, infarct intracranial hemorrhage, and/or subdural fluid collection. 12 Patients with SLE are commonly on immunosuppressive agents, and some patients have renal involvement leading to hypertension, which in turn can lead to PRES. 12 Posterior reversible encephalopathy syndrome is characterized by reversible vasogenic edema on imaging, and it can be complicated by infarction, hemorrhage, and vascuolopathy.…”

Síndrome da encefalopatia posterior reversível (PRES) e lúpus eritematoso sistêmico: relato de dois casos

“…15 Recently, Balint syndrome (a disorder of inaccurate visually guided saccades, optic ataxia, and simultanagnosia) presented as PRES in a SLE patient. 16 Of note, two reports accounted for the occurrence of PRES in juvenile SLE.…”