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– The case history of a 36-year-old woman with a life-long history of attacks of high fever and lymph-node swelling is described. The diagnosis appears to be a syndrome which we have called ‘hyperimmunoglobulinaemia D and periodic fever’. Fourteen patients are known with this syndrome which differs from familial Mediterranean fever and familial Hibernian fever. The role of the high IgD and interleukin-1 in this syndrome is subject to speculation.