Departments of Paediatrics, Universiti Teknologi MARA (UiTM) Faculty of Medicine, Sg Buloh, Selangor.
Departments of Radiology, Universiti Teknologi MARA (UiTM) Faculty of Medicine, Sg Buloh, Selangor.
Turk J Pediatr. 2021;63(5):743-751. doi: 10.24953/turkjped.2021.05.002.
Neuropsychiatric lupus (NPSLE) serves as a marker of severe disease in children with juvenile onset systemic lupus erythematosus (JSLE). This study aims to characterise the clinical and imaging features at diagnosis; and outcomes after 12 months in Malaysian children with NPSLE.
A retrospective study of all NPSLE patients seen at the Pediatric Rheumatology Unit, Selayang Hospital from January 2004 to May 2017.
Twenty-eight (19.8%) of 141 JSLE patients had NPSLE with a median presenting age of 10 years (IQR 9 - 12), median follow-up of 7 years (IQR 4 - 11) and female: male ratio of 3.7:1. Twenty-three patients had single episodes of NPSLE and five patients had two distinct episodes each. The mean disease activity score (SLEDAI- 2K) was 24.9±11.8 at presentation with 81.8% having high disease activity (score > 12). Majority (60.6%) present with NPSLE within the first year of SLE diagnosis whilst the remainder occurred at a median of five years (IQR 3-7) post-SLE diagnosis. Majority (75.8%) had central nervous system (CNS) involvement commonly presenting with seizures, delirium and visual complaints whilst 24.2% had peripheral nervous system (PNS) involvement. Frequent accompanying features included hypocomplementemia, acute cutaneous lupus and lupus nephritis. Autoantibodies were common; ANA (100%), anti-dsDNA (78.8%) anti-RNP (39.4%) and anti-Sm (39.4%). Abnormalities were seen in 85.7% of the magnetic resonance imaging (MRI) studies performed, predominantly supratentorial white matter hyperintensities on T2 images whilst cerebrospinal fluid examination was normal in the majority. All patients with CNS involvement received corticosteroids with immunosuppressive therapy: Cyclophosphamide (20), Rituximab (2). Treatment for PNS involvement included corticosteroids with Azathioprine (6) or Mycophenolate mofetil (2). At 12 months post-NPSLE, majority (85.7%) recovered without any neurological sequelae.
Juvenile-onset NPSLE presents with a myriad of clinical features. It is associated with high disease activity and non-specific MRI features. With early diagnosis and treatment, the majority had good prognosis.
神经精神性狼疮（NPSLE）是儿童幼年特发性系统性红斑狼疮（JSLE）疾病严重程度的标志。本研究旨在描述马来西亚儿童 NPSLE 患者的临床和影像学特征；以及 12 个月后的结局。
对 2004 年 1 月至 2017 年 5 月在赛莱亚医院儿科风湿病科就诊的所有 NPSLE 患者进行回顾性研究。
141 例 JSLE 患者中有 28 例（19.8%）患有 NPSLE，中位发病年龄为 10 岁（IQR 9-12），中位随访时间为 7 年（IQR 4-11），女性：男性比例为 3.7:1。23 例患者出现单次 NPSLE 发作，5 例患者各出现 2 次发作。就诊时平均疾病活动评分（SLEDAI-2K）为 24.9±11.8，81.8%患者有高疾病活动（评分>12）。大多数（60.6%）在 SLE 诊断后的 1 年内出现 NPSLE，其余患者则在 SLE 诊断后的中位时间 5 年（IQR 3-7）出现。大多数（75.8%）患者中枢神经系统（CNS）受累，常见表现为癫痫发作、意识障碍和视觉异常，而 24.2%患者外周神经系统（PNS）受累。常见的伴随特征包括低补体血症、急性皮肤狼疮和狼疮肾炎。自身抗体常见；ANA（100%）、抗 dsDNA（78.8%）、抗 RNP（39.4%）和抗 Sm（39.4%）。进行的 85.7%磁共振成像（MRI）检查显示异常，主要为 T2 图像上的幕上脑白质高信号，而大多数患者的脑脊液检查正常。所有 CNS 受累患者均接受皮质类固醇治疗，并接受免疫抑制治疗：环磷酰胺（20 例）、利妥昔单抗（2 例）。PNS 受累的治疗包括皮质类固醇治疗，阿扎胞苷（6 例）或霉酚酸酯（2 例）。在出现 NPSLE 后的 12 个月，大多数（85.7%）患者无任何神经后遗症而康复。
幼年起病的 NPSLE 表现出多种临床特征。它与高疾病活动度和非特异性 MRI 特征相关。早期诊断和治疗后，大多数患者预后良好。
