Clinical characteristics and efficacy of intrathecal therapy in pediatric lupus encephalopathy

Abstract

Background

To summarize the clinical characteristics of pediatric lupus encephalopathy and to investigate the therapeutic efficacy of intrathecal methotrexate and dexamethasone in the treatment of pediatric lupus encephalopathy.

Methods

A retrospective study was conducted on 83 children diagnosed with Neuropsychiatric systemic lupus erythematosus (NPSLE) at the Department of Pediatric Nephrology and Rheumatology of the First Affiliated Hospital of Sun Yat-sen University from January 2002 to December 2023. The intrathecal injection and non-injection groups were divided based on whether they received intrathecal injections of methotrexate and dexamethasone. Clinical symptoms, laboratory tests, renal biopsy pathology, disease activity, and treatments were compared between the two groups, and the efficacy of intrathecal injection therapy for NPSLE was also assessed.

Results

Of the 83 children with NPSLE, 14 were male and 69 were female. NPSLE was the initial manifestation in 12 (14.46%) patients, while 71 (85.54%) developed it after systemic lupus erythematosus onset. The most frequently observed symptoms were headache and seizures. Imaging (CT/MRI) in 81 children showed abnormalities in 64 (79.01%), with cerebral atrophy being most common. The results of electroencephalography in 21 patients demonstrated abnormalities in 14 cases, and 7 of the 29 patients exhibited abnormal cerebrospinal fluid findings. A total of 68.67% of NPSLE patients were classified as chronic kidney disease (CKD) stage 1, while 31.33% were CKD stage 2 or higher. Renal biopsy in 60 children commonly indicated class IV or IV+V. The SLEDAI score at initial consultation was 20.93 ± 6.41. Among the 83 patients, 10 (12.05%) received intrathecal injections with an average of 5.2 per patient. Before treatment, the injection group had higher SLEDAI scores (p < 0.05). After treatment, the resolution times for NPSLE-related symptoms and imaging were shorter in the injection group, although the difference did not reach statistical significance. Notably, the injection group had a lower SLEDAI score and a more pronounced reduction (p < 0.05).

Conclusion

Children with NPSLE in our center demonstrated more severe disease and higher disease activity index. Methotrexate and dexamethasone intrathecal therapy provided faster symptomatic relief and reduced disease activity in children with NPSLE.