Çocuklarda sistemik lupus eritematozusun klinik özellikleri ve uzun dönem sonuçları

Abstract

Background: Systemic lupus erythematosus (SLE) is a common multisystemic autoimmune disease characterized by the presence of autoantibodies and multiorgan system involvement. The aim of this study was to describe the presenting clinical manifestations, laboratuary findings, clinical course and prognosis of SLE in children. Methods: We performed a retrospective study patients with SLE, diagnosed before the age of 18 years. Clinical and laboratory data were collected from initial admission to study initiation. Results: Thirty-five children and adolescents SLE (cSLE) were recorded, 85.7% of female. The median age at disease onset was 12(range 4-17) years, and median follow up duration was 5 (1-14) years. The most common clinical feature was arthritis (65.1%), followed by constitutional symptoms (48.6%), malar rash (31.4%), photosensitivity (5.7%), alopecia (5.7%) and oral ulcers (5.7%). Renal involvement accounted 4/5 of the patients (80%). Hematuria and proteinuria were the most frequent presenting findings (48.5% and 45.7% respectively). Renal biopsy was performed in 27 patients. According to WHO classification: 1 patient had classV nephritis, 10 had class IV, 4 had class III and 12 had class II nephritis. 20% of children developed neurologic symptoms. One patient died during the follow-up period. At the last follow up none of the patients had renal failure but, proteinuria persisted in 4 of them (11.4%). All neurological findings of patients are in remission apart from sequelae. Conclusion: Clinical outcome was favorable in our patients. Renal involvement is common but progression to end stage kidney disease, at least in the short term, is rare.