Background <p>Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical and radiographic condition characterised by reversible vasogenic oedema in the brain. This condition is mainly caused by severe hypertension in the presence of underlying risk factors such as organ transplantation, certain medications, and autoimmune diseases, including systemic lupus erythematosus (SLE). Haemorrhagic complications associated with PRES are rare, mostly occurring after transplantation and are even less common in patients with a background of SLE.</p> Cases <p>We reported two cases diagnosed as active SLE with multisystem involvement. They presented with acute onset generalised tonic-clonic seizures, severe headache, and hypertensive emergency in the background of biopsy-proven lupus nephritis. MRI of the brain revealed vasogenic oedema involving the posterior aspect of the brain with haemorrhagic transformation. Both patients required prolonged intensive care admission with antihypertensive, immunosuppressive, and other supportive therapies. One of them (case 2) showed significant clinical and radiographic recovery. The other patient (case 1), although showing partial recovery in the initial phase, had ongoing sepsis and eventually succumbed.</p> Conclusion <p>Haemorrhagic conversion of PRES in the context of SLE is rarely reported. To date, fewer than 10 case series have been reported with SLE-related haemorrhagic PRES, and these two cases emphasise the significance of this entity in the context of lupus. A detailed review highlights the comparative characteristics of our cases and those reported in the literature.</p>

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A Two-Case Series and Literature Review of Unusual Haemorrhagic Presentation of Posterior Reversible Encephalopathy Syndrome (PRES) in Systemic Lupus Erythematosus

  • Sourav Garai,
  • Ritasman Baisya,
  • Sukdev Manna,
  • Soumya Bhowmik,
  • Sneha Dhali,
  • Swarnava Tarafdar

摘要

Background

Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical and radiographic condition characterised by reversible vasogenic oedema in the brain. This condition is mainly caused by severe hypertension in the presence of underlying risk factors such as organ transplantation, certain medications, and autoimmune diseases, including systemic lupus erythematosus (SLE). Haemorrhagic complications associated with PRES are rare, mostly occurring after transplantation and are even less common in patients with a background of SLE.

Cases

We reported two cases diagnosed as active SLE with multisystem involvement. They presented with acute onset generalised tonic-clonic seizures, severe headache, and hypertensive emergency in the background of biopsy-proven lupus nephritis. MRI of the brain revealed vasogenic oedema involving the posterior aspect of the brain with haemorrhagic transformation. Both patients required prolonged intensive care admission with antihypertensive, immunosuppressive, and other supportive therapies. One of them (case 2) showed significant clinical and radiographic recovery. The other patient (case 1), although showing partial recovery in the initial phase, had ongoing sepsis and eventually succumbed.

Conclusion

Haemorrhagic conversion of PRES in the context of SLE is rarely reported. To date, fewer than 10 case series have been reported with SLE-related haemorrhagic PRES, and these two cases emphasise the significance of this entity in the context of lupus. A detailed review highlights the comparative characteristics of our cases and those reported in the literature.