An unusual case of familial Mediterranean fever.
Familial Mediterranean fever (FMF) is an inherited disorder characterized by recurrent self-limiting attacks of joint, chest and abdominal pain associated with fever. The most serious complication in FMF is the development of amyloidosis, which usually leads to death from renal failure within a year. The use of colchicine has dramatically reduced this complication. We describe a 56-yr-old female patient with FMF in whom the arthropathy became the dominant clinical feature, resulting in the development of an erosive large and small joint arthritis during the course of the disease. The patient was treated with colchicine, but despite this, later developed amyloidosis confirmed on rectal biopsy, and chlorambucil was added to her treatment. For 10 yr, she also suffered intermittent abdominal pain and had terminal ileal changes suggestive of Crohn's disease. However, she was found to have ischaemic colitis at post mortem secondary to amyloidosis. Ischaemic bowel disease is an extremely unusual event in FMF. Other factors which may have contributed to the terminal ischaemia in this patient include anaemia secondary to blood loss and a drug-induced myelodysplasia, as well as hypotension during the final septicaemic illness. Clinicians should consider an ischaemic colitis as a possible differential diagnosis of abdominal pain in patients with FMF even in the absence of other clinical evidence of systemic amyloidosis.
Steuer
A
A
Department of Rheumatology, Northwick Park and St Mark's Hospital, Harrow.
Leonard
N
N
Ahmed
F B
FB
Price
A B
AB
Gumpel
J M
JM
D015746:Abdominal Pain / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D000686:Amyloidosis / Q000150:complications / Q000188:drug therapy / Q000209:etiology; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D001168:Arthritis / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D002699:Chlorambucil / Q000627:therapeutic use; D003078:Colchicine / Q000627:therapeutic use; D017091:Colitis, Ischemic / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D006801:Humans; D007082:Ileum / Q000473:pathology; D008875:Middle Aged; D011859:Radiography; D051437:Renal Insufficiency / Q000209:etiology / Q000473:pathology
D000893:Anti-Inflammatory Agents; D018906:Antineoplastic Agents, Alkylating; D002699:Chlorambucil; D003078:Colchicine