A retrospective review of patients was conducted to evaluate the short-term effect of therapeutic plasma exchange (TPE) in children with active systemic lupus erythematosus (SLE). Between April 1984 and March 2003, 22 children underwent 112 TPE sessions. The median number of daily, double-volume sessions was 6 (range 1-16). Plasma exchange was performed by centrifugation, using 4.5% albumin with fresh-frozen plasma as required. All children were receiving standard immunosuppressive therapy. The predominant clinical features that led to TPE were categorised into systems and outcome was evaluated according to clinical improvement or lack of it. There was benefit for central nervous system and pulmonary manifestations (10 cases, 11 episodes). Of 8 children treated for renal manifestations, 6 achieved good outcomes. Two children became dialysis dependent despite treatment; however, both had been successfully treated with TPE before. Five children received more than one course of TPE. No child died during the procedure and major complications were few. Our data show that TPE can be used as adjunctive therapy in childhood SLE with few complications. Outcome is difficult to measure but in our experience, during the acute phase of illness, TPE appears to be of benefit.