Perfil epidemiológico, clínico e terapêutico de pacientes com lúpus eritematoso sistêmico juvenil em um hospital terciário.

Abstract

Juvenile systemic lupus erythematosus (JSLE) is a chronic inflammatory disease, of unknown cause and autoimmune nature, which starts early, under the age of 18 years. Between 10 and 20% of the cases, can start in children and adolescents, when it is associated with worse outcomes, with a predominance of severe impairment. The objective of this study was to characterize the clinical, epidemiological and therapeutic profile of patients with JSLE at a reference hospital in the interior of the Brazilian Northeast, through a descriptive and sectional study, using data obtained from medical records of 34 patients with a suspected diagnosis or confirmed by LESJ, between the months of June 2007 and June 2017. In the period of analysis, there was a predominance of female, black, brown and adolescent patients, with a mean age of onset of the disease of 14 ± 2.5 years. The most common clinical manifestation was non-erosive arthritis, observed in 64.7% of cases, followed by erythema malar, present in 55.8%. Haematological and renal changes were observed, respectively, in 56% and 50% of the patients. Neuropsychiatric manifestations were seen in 14.7% of the cases, including one case of catatonia and another case of chorea, rare in SLE. Renal biopsy was performed only 29.4% of the cases of renal involvement. Four deaths were recorded, all due to septic shock, which is equivalent to a mortality rate of 11.8%. The FAN-HEp2, positive in 88.2% of the cases, had as predominant pattern the fine nuclear dotted. Autoantibodies were observed in 47% of cases. The use of oral corticoids was instituted for all and hydroxychloroquine was administered to 88.3% of the patients. This sample comprised preponderantly female adolescents, black and brown, with cutaneomucous, osteoarticular and hematological alterations. Renal impairment was also frequent and associated with severe forms of the disease, as well as with the evolution to death. The incidence rate was higher than that observed in the literature, which reinforces the need for SLE to suspect and treat early.