Posterior Reversible Encephalopathy Syndrome–An Insidious Manifestation of Systemic Lupus Erythematosus

1North Shore-LIJ, Manhasset, NY

Meeting: Hospital Medicine 2015, March 29-April 1, National Harbor, Md.

Abstract number: 548


Case Presentation: A thirty-five year old African-American female with a history Multiple Sclerosis and uterine fibroids presented with anemia, acute kidney injury (AKI), and lower extremity edema for two weeks. Initially AKI was attributed to obstructive uropathy secondary to massive fibroids affecting both kidneys, with atrophic R kidney seen on imaging. However, when AKI began worsening after myomectomy and left ureteral stent placement, and proteinuria progressed to greater than eleven grams, an alternate etiology was investigated. Subsequent serologies were consistent with the diagnosis of SLE. Patient was started on corticosteroids and Mycophenolate Mofetil for presumed lupus nephritis (renal biopsy was considered high risk). Meanwhile, the patient developed confusion, visual hallucinations, and agitation with systolic BP peaking in the low 200’s and diastolic in the 110’s. The next day the patient became unresponsive, required endotracheal intubation, and was found to have seizure activity on EEG. At this time our differential included multiple sclerosis flare, steroid induced psychosis, neuropsychiatric lupus, stroke, and PRES. MRI showed new edema involving the left pre and postcentral gyri, and bilateral occipital poles, consistent with PRES. Based on correlation of her neurological symptoms with hypertension rather than the initiation of immunomodulators, it was felt that PRES was secondary to uncontrolled hypertension. Patient’s blood pressure was tightly regulated with a goal systolic BP < 140 and diastolic <90. After a few days there was an improvement in the patient’s mental status. Repeat MRI two weeks later showed resolution of radiological findings. 

Discussion: Posterior reversible encephalopathy syndrome (PRES) is an under recognized (only 109 reported cases in the English literature to date upon review) and reversible condition seen in Systemic Lupus Erythematosus. Clinically, neuropsychiatric lupus can resemble PRES, posing diagnostic and therapeutic challenges. PRES is characterized by seizures, altered mental state, and visual disturbances. It has been associated with acute hypertension, nephritis, pre-eclampsia, and immunosupressive therapies. Treatment involves treating the underlying cause. Most individuals exhibit complete neurologic recovery within 1-4 weeks, with resolution of radiologic lesions. Therapeutic implications of distinguishing between the underlying etiologies are significant. In this case, PRES was attributed to hypertension and renal failure due to lupus nephritis, thus immunosuppressants and corticosteroids were continued. This allowed us to continue to treat the underlying Lupus nephritis and prevent associated complications.  

Conclusions: PRES is a life threatening yet treatable condition. Prompt diagnosis and treatment is essential in order to minimize the life threatening complications associated with the disease and to prevent the use of toxic treatments given to those suspected to have mimicking conditions.

To cite this abstract:

Jain P. Posterior Reversible Encephalopathy Syndrome–An Insidious Manifestation of Systemic Lupus Erythematosus. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 548. Journal of Hospital Medicine. 2015; 10 (suppl 2). Accessed March 31, 2020.

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