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Abstract Introduction: Systemic lupus erythematosus (SLE) is a multisystem autoimmune connective tissue disease, 20% of cases having their onset in childhood and are classified as juvenile-onset SLE (JSLE). Objective: The aim of this work is to assess the basophilic count as a marker for disease activity in JSLE to enable the earlier diagnosis and treatment of SLE flares. Patients and methods: This cross-sectional cohort study was conducted on 80 children with JSLE following at pediatric rheumatology clinic, Specialized Children{u2019}s hospital, Cairo University and compared to 100 healthy children as a control group. SLEDAI-2K score was done at time of the study, together with CBC with differential to evaluate basophilic count. Results: The mean age of disease onset was 9.14 ± 2.23 years. Among the clinical manifestations at presentation, arthritis and malar rash (37.5% each) were the most common. Throughout the course of the disease malar rash (70%) and nephritis (67.5%) were the most common disease manifestations. Thirty-eight patients (70.3%) did renal biopsy to diagnose nephritis. Basophil counts were similar in patients with SLE and healthy controls (0.0±0.04x109/L), p value =0.06) and was within normal range. Basophils absolute count was within normal range (0 - 0.1x 109 /L) in most of our patients. There was relative basophilia in active patients{u2019} group which increased with higher SLEDAI-2K score but with no statistical significance basophil counts were (0.01± 0.03x109/L) in SLEDAI-2K score (4-8) and were (0.03±0.07X109/L) in SLEDAI-2K score more than 8 |