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This challenges optimal management when the aetiology is non-rheumatic, as exemplified below in an 8-year-old girl, hailing from Uganda, who was diagnosed and managed as rheumatic MR with unusually elevated and persistent systemic inflammatory state with chronic renal dysfunction disproportionate to her cardiac status. Further workup led towards the possibility of early onset systemic lupus erythematosus (SLE). The valvular involvement in SLE closely resembles rheumatic heart disease (RHD). A high level of clinical suspicion is essential for diagnosing childhood-onset SLE. 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