BIOLOGIC AND CLINICAL ADVANCES IN FAMILIAL MEDITERRANEAN FEVER

Familial Mediterranean fever (FMF) is an inherited disease manifested by acute but reversible attacks of sterile inflammation affecting serosal and synovial spaces. The encoding gene for the disease is located on the short arm of chromosome 16. Patients from certain ethnic groups tend to develop amyloidosis, clinically manifested by nephropathy. Both clinical syndromes can be prevented by prophylactic treatment with colchicine. The cause of the inflammatory attacks in FMF appears to be an inherited deficiency of a C5a/IL-8 inactivating protease in the serosal tissues. © 1995.