# Juvenile systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome: a case report and literature review

**Authors:** Man Luo, Huan He, Qing Zhou, Long Chen, Changming Xia, GuoHua Yuan, Fang He

PMC · DOI: 10.1186/s13023-025-04044-0 · Orphanet Journal of Rare Diseases · 2025-11-07

## TL;DR

A child with lupus developed a rare brain condition called PRES, which improved with treatment, and similar cases often involve kidney issues and high disease activity.

## Contribution

This case report adds to the understanding of PRES in juvenile SLE by highlighting its association with lupus nephritis and high disease activity.

## Key findings

- The patient's PRES symptoms resolved after treatment with blood pressure control and immunosuppressants.
- Review of 15 cases showed most children with SLE and PRES recovered with active treatment.
- Juvenile SLE with PRES is often linked to lupus nephritis, hypertension, and high disease activity.

## Abstract

Posterior reversible encephalopathy syndrome (PRES) is an acute or subacute neurological syndrome with clinical manifestations including headache, vision loss, altered mental status, and seizures. Magnetic resonance imaging(MRI) can assist in clarifying the diagnosis. Systemic lupus erythematosus (SLE) is an autoimmune disease, and the occurrence of PRES is infrequent, especially in children with SLE.

Our patient was initially diagnosed with juvenile systemic lupus erythematosus involving the skin, joints, and kidneys. The patient was treated with glucocorticoids and cyclophosphamide, which led to a partial improvement in symptoms. However, the patient experienced neurological symptoms and elevated blood pressure, and head MRI revealed swelling and diffuse damage to the bilateral occipital parietal gyrus. The patient was diagnosed with PRES.

The symptoms were relieved after actively lowering blood pressure, dehydrating with mannitol, and treating with glucocorticoids, cyclophosphamide, and telitacicept. Six months later, no abnormalities were observed in the follow-up head MR image. We searched PubMed to review the characteristics of 15 children with SLE who developed PRES, and most of them recovered after active treatment.

Various factors can lead to complications of PRES in paediatric patients with systemic lupus erythematosus. In our patient, the likely cause was the high disease activity of SLE. This review revealed that juvenile SLE cases accompanied by PRES are often associated with lupus nephritis, hypertension, and high disease activity. Most patients recover with timely and active treatment, with only a few experiencing recurrence.

## Linked entities

- **Chemicals:** mannitol (PubChem CID 6251), cyclophosphamide (PubChem CID 2907)
- **Diseases:** systemic lupus erythematosus (MONDO:0007915), lupus nephritis (MONDO:0005556), posterior reversible encephalopathy syndrome (MONDO:0044033)

## Full-text entities

- **Diseases:** swelling (MESH:D004487), lupus nephritis (MESH:D008181), autoimmune disease (MESH:D001327), hypertension (MESH:D006973), headache (MESH:D006261), Juvenile systemic lupus erythematosus (MESH:D008180), PRES (MESH:D054038), vision loss (MESH:D014786), seizures (MESH:D012640), neurological syndrome (MESH:D009461)
- **Chemicals:** cyclophosphamide (MESH:D003520), mannitol (MESH:D008353)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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Source: https://tomesphere.com/paper/PMC12595737