Background. The reactive haemophagocytic syndrome (RHS) is a little‐known life‐threatening complication of rheumatic diseases in children. It reflects the extreme vulnerability of these patients, especially those with systemic‐onset juvenile chronic arthritis (JCA). This immunohaematological process may be triggered by events such as herpes virus infection and non‐steroidal anti‐inflammatory drug therapy. Treatment has not been standardized.