Department of Clinical Immunology and Rheumatology, 28605Nizam's Institute of Medical Sciences (NIMS), Hyderabad, India.
Lupus. 2022 Apr;31(5):606-612. doi: 10.1177/09612033221088207. Epub 2022 Mar 25.
PRES, as a complication of juvenile lupus, is rarely reported in the literature. In this study, six juvenile lupus patients admitted with diagnosis of PRES were assessed on the basis of clinical characteristics, imaging findings, disease activity status, treatment response and prognosis.
Six juvenile (≤ 16 years) lupus patients with a diagnosis of PRES were included. Demographic, clinical, and laboratory features and outcomes of all six patients were noted. Literature review was performed on PubMed search forum. Search terms in English included Juvenile SLE, Lupus and PRES.
The youngest patient was seven years old while the oldest was sixteen years. All patients had history of lupus nephritis , presented with seizure and hypertension. In imaging, four out of six patients had hyperintensities in atypical distribution suggesting atypical PRES. All the patients had significant clinical recovery with resolution of hyperintensities in five out of six patients on repeat imaging.
Juvenile lupus with PRES is considered an unusual neurologic manifestation triggered by multiple factors. It can be stipulated that PRES in juvenile lupus cases often remain undiagnosed. Early suspicion and treatment institution with reversal of triggers can result in a favorable outcome in these patients.
作为青少年狼疮的一种并发症， PRES 很少在文献中报道。在这项研究中，根据临床特征、影像学表现、疾病活动状态、治疗反应和预后，对 6 名被诊断为 PRES 的青少年狼疮患者进行了评估。
纳入了 6 名（≤16 岁）被诊断为 PRES 的青少年狼疮患者。记录了所有 6 名患者的人口统计学、临床和实验室特征及结局。在 PubMed 检索论坛上进行了英文文献回顾。英文检索词包括青少年系统性红斑狼疮、狼疮和 PRES。
最小的患者为 7 岁，最大的为 16 岁。所有患者均有狼疮肾炎病史，表现为癫痫发作和高血压。在影像学上，6 名患者中有 4 名出现非典型分布的高信号，提示为非典型 PRES。所有患者均有显著的临床恢复，6 名患者中有 5 名在重复影像学检查时高信号消失。
PRES 是青少年狼疮一种不常见的神经表现，由多种因素引发。可以认为，青少年狼疮患者的 PRES 往往未被诊断。早期怀疑和治疗诱因可以使这些患者获得良好的结局。
