Familial Mediterranean fever (FMF) is an inherited disorder of unknown etiology characterized by recurrent episodes of serous membrane inflammation. Interleukin 1 (IL-1) is a mediator of inflammatory processes. We hypothesized that IL-1 may play a role in acute attacks of FMF. Thus we tested IL-1 production by monocytes derived from patients with FMF. Nine patients were tested during acute attacks and 9 were asymptomatic when tested. Monocytes derived from peripheral blood of patients and controls were stimulated with lipopolysaccharide (LPS) and IL-1 activity in the supernatant was tested using a T helper cell line (D10-4G.1). IL-1 secretion during acute attacks was decreased whereas IL-1 production in asymptomatic patients was comparable to healthy controls. Followup of symptomatic patients during the recovery period revealed normalization of IL-1 secretion. Addition of indomethacin (prostaglandin E2 inhibitor) to LPS stimulated monocytes did not change IL-1 activity in patients or healthy controls. We conclude that in vitro IL-1 activity in patients with FMF is associated with the intensity of the inflammatory process.