Consecutive PRES and diffuse alveolar hemorrhage in a patient with active lupus nephritis and antiphospholipid antibodies: a case report and systematic literature review

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but potentially life-threatening neurological complication of systemic lupus erythematosus (SLE). Its pathogenesis is multifactorial, especially in patients with active lupus nephritis receiving intensive immunosuppression. We report a 24-year-old woman with active class III/V lupus nephritis, persistent antiphospholipid antibody positivity, and profound hypoalbuminemia who developed severe atypical PRES with extensive multifocal vasogenic edema, despite only mildly elevated blood pressure, followed within 48 hours by fatal diffuse alveolar hemorrhage. A systematic PubMed search identified original case reports and case series of PRES in SLE. To avoid duplication, only studies with extractable individual patient data were included. Sixty-three studies comprising 103 patients were analyzed. PRES predominantly affected young women (93.3%), with a mean age of 27.3 years. Immunosuppressive therapy was a major precipitating factor, with corticosteroids implicated in 45.7% and cyclophosphamide in 14.3% of cases. Renal involvement, high disease activity, and endothelial dysfunction were frequent coexisting features. These findings suggest that cyclophosphamide usually acts as a final trigger on a critically primed endothelium rather than an isolated cause. Profound hypoalbuminemia and nephrotic syndrome may increase vulnerability by destabilizing the blood–brain barrier. Although often reversible, PRES may herald fatal multisystem endothelial failure in high-risk patients, underscoring the need for early recognition and multidisciplinary management.