Familial Mediterranean Fever

FMF is a rare autosomal recessive disorder characterized by paroxysms of fever and serosal inflammation, seen primarily in several ethnic groups originating in the Mediterranean region. Typical clinical presentations of the disease are recurrent attacks of severe pain (due to serositis at one or more sites) and fever, lasting one to three days, and then resolving spontaneously. In between attacks, patients are entirely well.

Common manifestation are peritonitis, pleuritis, synovitis and an erysipelas-like skin lesion. Other less common findings are pericarditis, orchitis and recurrent aseptic meningitis also can occur. As was mentioned this morning, an increased incidence of some vasculitides, such as polyarteritis nodosa and Henoch-Schönlein purpura, has been described. Kidney involvement with these processes may be particularly common.

As discussed this morning, the most important long term complication of FMF is secondary (AA) amyloidosis which occurs insidiously and progressively. Amyloid A deposition occurs in the kidney, spleen, liver, and gut. Renal involvement is the dominant feature of FMF-related amyloidosis.

Colchicine is the mainstay of treatment of FMF, both to prevent attacks as well as prevent the development and progression of amyloidosis. Research is underway evaluating the role of anti-TNF-alpha therapy and IL-1 receptor blockade in severe FMF.

Here two good articles are treatment and clinical manifestations of FMF if you're interested.
http://www.ncbi.nlm.nih.gov.myaccess.library.utoronto.ca/pubmed/19530512
Familial Mediterranean fever. Ben-Chetrit E, Levy M. Lancet. 1998;351(9103):659.