Neuropsychiatric Syndromes in Childhood-Onset Systemic Lupus Erythematosus: A Systematic Review and Meta-analysis.

OBJECTIVE

The aim of this study was to access the prevalence of 19 neuropsychiatric syndromes in childhood-onset systemic lupus erythematosus (cSLE), as defined by the American College of Rheumatology (ACR) in 1999, by performing a systematic review and meta-analysis of relevant publications.

METHODS

A literature search from April 1999 to March 2018 identified studies investigating neuropsychiatric syndromes in cSLE patients, applying 1999 ACR Case Definitions, with a sample of at least 20 patients. Case reports, small case series, reviews, articles that did not use 1999 ACR case definitions, and those with adult SLE patients were excluded. The methodological quality of the studies was determined through the Loney quality assessment. Prevalence estimates with a 95% confidence interval were combined using random-effect (DerSimonian-Laird) models.

RESULTS

A total of 143 articles were identified and 9 were included. In a population of 1463 cSLE patients, 351 (29.9%) presented 869 neuropsychiatric cSLE events (2.48 events/patient). The results for each syndrome were headache (52.2%), seizure disorders (48.6%), cognitive dysfunction (32.9%), mood disorder (28.3%), psychosis (22.7%), cerebrovascular disease (19.5%), acute confusional state (15.7%), movement disorder (9.4%), anxiety disorder (7.2%), aseptic meningitis (5.1%), mononeuropathy single/multiplex (4.9%), myelopathy (4.2%), demyelinating syndrome (3.2%), cranial neuropathy (2.7%), polyneuropathy (2.6%), Guillain-Barré syndrome (2.5%), autonomic disorder (1.9%), plexopathy (1.3%), and myasthenia gravis (1.3%).

CONCLUSIONS

Neuropsychiatric syndromes are prevalent among cSLE patients. The most prevalent were headaches, seizures, cognitive dysfunction, mood disorders, and psychosis. Determining the prevalence of each neuropsychiatric syndrome in cSLE may improve clinical awareness of these potentially fatal and disabling conditions.