Orphanet Journal of Rare Diseases | A Case Analysis and Literature Review of Pediatric Systemic Lupus Erythematosus Complicated with PRES

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This article reports a rare case of pediatric systemic lupus erythematosus (SLE) complicated with posterior reversible encephalopathy syndrome (PRES), and analyzes its precipitating factors and clinical features in combination with a literature review, providing important references for early diagnosis and intervention.

 

Literature Overview
The article titled Juvenile systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome: a case report and literature review was published in the Orphanet Journal of Rare Diseases, summarizing clinical data from 16 cases of pediatric SLE complicated with PRES. The study indicates that PRES is uncommon in SLE patients and even rarer in the pediatric population. Major precipitating factors include lupus nephritis, hypertension, high disease activity, and the use of immunosuppressive agents. The article further analyzes the imaging manifestations, treatment strategies, and prognosis of these cases.

Background Knowledge
Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder with particularly high disease activity in children. Posterior reversible encephalopathy syndrome (PRES) is a neuroimaging syndrome characterized by acute or subacute encephalopathy, commonly observed in conditions such as hypertension, renal insufficiency, and immunosuppressive therapy. Its typical MRI feature is vasogenic brain edema predominantly affecting the parieto-occipital regions. While PRES has been widely reported in adult SLE patients, studies in children remain limited, and its exact pathogenesis remains unclear. The prevailing hypotheses include hypertensive breakthrough of cerebral autoregulation, immune-mediated endothelial injury, drug toxicity, and cytokine release. Pediatric SLE cases complicated with PRES usually present with severe systemic inflammation and multi-organ involvement, especially lupus nephritis (LN) and hypertension, suggesting that high disease activity may be a key trigger. Due to the lower cerebral autoregulation threshold in children, PRES may develop even without significant blood pressure elevation. This study provides a systematic analysis of clinical features, imaging manifestations, and treatment strategies of SLE-related PRES in children for clinicians.

 

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Research Methods and Experiments
The research team reported a case of PRES in a 10-year-old girl with systemic lupus erythematosus. Through a PubMed database search, they reviewed 15 additional pediatric cases of SLE with PRES reported in the literature. Imaging data included findings from head CT and MRI, while clinical data encompassed disease activity scores (SLEDAI), blood pressure, laboratory tests, and treatment regimens. The researchers conducted a systematic analysis of the clinical features, precipitating factors, imaging characteristics, and treatment outcomes of PRES.

Key Conclusions and Perspectives

• Cases of pediatric SLE complicated with PRES are rare, but when they occur, major precipitating factors include lupus nephritis, hypertension, and high disease activity.
• The main clinical manifestations include headache, seizures, visual disturbances, and altered consciousness; all cases presented with seizures.
• Imaging features are predominantly in the parieto-occipital regions, with some cases involving the temporal lobes, frontal lobes, cerebellum, and basal ganglia.
• Most patients showed good recovery after active treatment, although a minority experienced recurrence or neurological sequelae.
• In this case, symptoms improved after treatment with methylprednisolone, cyclophosphamide, and telitacicept, with normalization of MRI findings, suggesting that treating SLE may have a positive effect on PRES.

Research Implications and Future Directions
This study highlights the importance of identifying PRES in pediatric SLE patients, especially in those with lupus nephritis and hypertension. Early diagnosis and treatment can significantly improve prognosis and prevent serious complications such as cerebral hemorrhage or infarction. Further research is needed to clarify the exact pathogenesis of PRES in pediatric SLE, particularly the role of inflammatory cytokines in endothelial injury, to develop more precise preventive and therapeutic strategies.

 

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Conclusion
Through a systematic review of 16 pediatric SLE cases complicated with PRES, this study confirms the rarity of this syndrome in children and identifies its major triggers, including lupus nephritis, hypertension, and high disease activity. All cases showed seizures, and MRI predominantly revealed parieto-occipital edema. The study suggests that PRES in children may not be entirely dependent on severe hypertension, but rather closely related to systemic inflammatory status. Treatment strategies including blood pressure control, intracranial pressure reduction, and aggressive SLE management are beneficial for recovery. This article serves as an important reference for clinicians in identifying and managing neurological complications in pediatric SLE patients, emphasizing the necessity of early imaging evaluation, especially when neurological symptoms arise. Although most patients have favorable outcomes, delayed diagnosis may lead to irreversible neurological damage, underscoring the need for increased awareness of this syndrome.

 

Man Luo, Huan He, Qing Zhou, GuoHua Yuan, and Fang He. Juvenile systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome: a case report and literature review. Orphanet Journal of Rare Diseases.