Posterior reversible encephalopathy syndrome in the setting of severe systemic lupus erythematosus activity with a rapidly fatal outcome: a case report.

BACKGROUND

Posterior reversible encephalopathy syndrome (PRES) is typically considered a self-limiting neurological disorder with a favorable prognosis when promptly diagnosed and treated. However, we report a rare fulminant case of PRES in a patient with systemic lupus erythematosus (SLE), challenging the notion of its uniformly benign course. CASE PRESENTATION: A 50-year-old Asian woman with a recent diagnosis of active SLE and dialysis-dependent renal failure presented with an 8-hour history of headache, dizziness, visual impairment, and seizures. Brain MRI revealed bilateral parieto-occipital lesions consistent with PRES. Diffusion-weighted imaging (DWI) showed mixed hyperintensity corresponding to low signal on the apparent diffusion coefficient (ADC) map, confirming true diffusion restriction and indicating cytotoxic edema. Despite immediate admission to the neurological intensive care unit and administration of corticosteroids, antihypertensives, and antiepileptics, the patient experienced rapid neurological deterioration. Within 70 min, she developed massive oral and nasal bleeding, followed by cardiac arrest and death. Laboratory tests showed moderate thrombocytopenia, but no platelet transfusion was administered per current guidelines due to the absence of active bleeding at the time of admission.

CONCLUSIONS

This case highlights a fulminant and multifactorial clinical course of severe systemic lupus erythematosus, in which PRES represented a secondary neurological manifestation rather than the primary cause of death. It underscores the importance of recognizing PRES in patients with active SLE as a marker of severe systemic disease activity and emphasizes the need for prompt multidisciplinary evaluation and comprehensive management of underlying systemic involvement.