Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by the production of various auto antibodies including anti-nuclear antibody, double stranded DNA and antibodies to extractable nuclear antigens (ENA), which include four groups of RNA binding protein namely Sm, RNP, SSA/Ro and SSB/La. The objectives were to detect the frequency and pattern of ANA, frequency of anti-ENA antibodies and association between the clinical presentations with ANA and Anti-ENA antibodies in paediatric SLE patients. It was a cross sectional study and the data was collected from patients fulfilling the ACR 1997 revised criteria of SLE in the Department of Paediatrics, BSMMU, Dhaka, Bangladesh. Among 50 paediatric SLE patients, ANA was positive in 94% of the patients. Homogenous pattern of ANA was the highest (57%) followed by speckled (31%), cytoplasmic (6.6%) and nucleolar (3.3%) patterns. Anti-ds DNA positivity was 86% and anti-ENA positivity was 56% in this study. Among them, anti-RNP was found in 43.5% followed by anti-sm (24%), anti-SSA (24%) and anti-SSB (17%). Anti-RNP antibody was associated with renal and gastrointestinal and Anti-SSA was associated with only gastrointestinal manifestations. ANA, anti ds-DNA, anti ENA positivity were found significantly among paediatric SLE patients and clinical presentation of them had association with Anti-ENA antibodies.

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