A case of familial Mediterranean fever with obvious family history

Abstract

A 26-year-old female was admitted to our hospital on December 4, 1992, because of recurrent fever. She had experienced recurrent fever of over 38 degrees C, occurring at irregular intervals 4-6 times a year with chest or abdominal pain, since the age of 19. After delivery of a baby at the age of 25, her symptoms had increased to once a week. In the febrile phase, leukocytosis, an increased erythrocyte sedimentation rate and positive CRP were recognized. These symptoms and laboratory findings spontaneously disappeared within a few days. Despite systemic and careful examinations, no evidence of infectious diseases, collagen diseases or malignant diseases were found. There were no significant differences of serum and urine catecholamines, and urine etiocholanolone between the febrile phase and the afebrile phase. An intravenous infusion of metaraminol induced symptoms similar to a spontaneous attack, and the metaraminol rechallenge test became negative after she was treated with oral colchicine. Based on these findings, she was diagnosed as having familial Mediterranean fever. Since she was treated with colchicine, the febrile attacks have decreased. Significantly, her elder brother has had similar recurrent fever with abdominal pain. He was diagnosed as having familial Mediterranean fever due to a positive metaraminol provocative test, and his febrile attacks have also been suppressed by colchicine. This is the first case of familial Mediterranean fever with obvious family history in Japan.