NEUROPSYCHIATRIC DISORDERS IN JUVENILE-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: RESULTS FROM A RETROSPECTIVE STUDY

Abstract

Objective: to analyze clinical features and immunological abnormalities in juvenile-onset systemic lupus erythematosus (SLE) with neurolupus and to compare findings with the data available in the literature.Subjects and methods. The observational retrospective study included 218 patients (190 girls and 28 boys) with juvenileonset SLE who were treated at the Pediatric Department of the V.A. Nasonova Research Institute of Rheumatology in the period from 1992 to 2017. The investigators assessed demographic parameters, the data of clinical and laboratoryinstrumental examinations, and the results of psychological, neurological and, if indicated, psychiatric examinations. The clinical and immunological features of SLE onset were comparatively analyzed in the groups of patients with and without neurolepus, followed by a comparison of the findings with the data available in the literature.Results and discussion. Forty-five (20.6%) patients with SLE and neuropsychiatric disorders were identified, of them there were 9 (20%) boys. In the neurolupus group, the mean age at onset was 13.0±2.8 years; the median disease duration at diagnosis verification was 5.0 [3.0; 11.0] months. 60% of patients were aged 10 to 15 years at diagnosis verification. Neurolupus was significantly more often detected in 46.7% of patients with acute SLE (p=0.003). Among the clinical manifestations of a nervous system lesion, serositis (p=0.003) and kidney disease (p=0.003) were diagnosed significantly more often; chronic skin changes (p=0.076) were recorded slightly more frequently, whereas arthritis (p=0.028) was detected significantly less frequently. Of the hematological disorders, leuko- and lymphopenia (p=0.087) and thrombocytopenia (p=0.077) were noted relatively more commonly. Of the immunological disorders, patients with nervous system lesion more frequently had anti-ribonucleoprotein antibodies (p=0.073) without any differences in other immunological parameters. In general, the neurolupus group showed a greater extent of multiple organ dysfunction than the other patients (the number of clinical manifestations averaged 5.6 and 3.7, respectively; p<0.0001).In all the patients, the manifestation of neurolupus was preceded by school maladaptation and emotional disturbances. There was a preponderance of central nervous system lesion (89%) in the pattern of neuropsychiatric manifestations. Fifteen (33.3%) patients had more than one manifestation of neurolupus. Among the neuropsychiatric disorders, there were headaches (28.9%), cognitive impairment (28.9%), cerebrovascular disease (35.5%), distal sensory polyneuropathy (20%), epilepsy syndrome (15.5%), anxiety disorders (11.1%), psychoses (8.9%), myelopathy (6.7%), and chorea (4.4%). In the neurolupus group, the SLEDAI scores were significantly higher (22.0±9.5) than in the non-neurolupus group (12.9±6.5; p<0.0001).Conclusion. When the patient has an acute onset, multiple organ dysfunction, psychological problems as emotional lability, proneness to conflict, and school maladaptation, he/she must undergo comprehensive examination to exclude neurolupus before the disease manifests. Identification of nervous system lesion requires urgent intensification of therapy to improve prognosis.

Keywords

• juvenile-onset systemic lupus erythematosus
• neurolupus in children
• childhood and adolescence