AN UNUSUAL CASE OF FAMILIAL MEDITERRANEAN FEVER

Familial Mediterranean fever (FMF) is an inherited disorder characteri zed by recurrent self-limiting attacks of joint, chest and abdominal p ain associated with fever. The most serious complication in FMF is the development of amyloidosis, which usually leads to death from renal f ailure within a year. The use of colchicine has dramatically reduced t his complication. We describe a 56-yr-old female patient with FMF in w hom the arthropathy became the dominant clinical feature, resulting in the development of an erosive large and small joint arthritis during the course of tile disease. The patient was treated with colchicine, b ut despite this, later developed amyloidosis confirmed on rectal biops y. and chlorambucil was added to her treatment. For 10 yr, she also su ffered intermittent abdominal pain and had terminal ileal changes sugg estive of Crohn's disease. However, she was found to have ischaemic co litis at post mortem secondary to amyloidosis. Ischaemic bowel disease is an extremely unusual event in FMF. Other factors which may have co ntributed to the terminal ischaemia in this patient include anaemia se condary to blood loss and a drug-induced myelodysplasia, as well as hy potension during the final septicaemic illness. Clinicians should cons ider an ischaemic colitis as a possible differential diagnosis of abdo minal pain in patients with FMF even in the absence of other clinical evidence of systemic amyloidosis.