[Familial Mediterranean fever with amyloidosis. Recent pathogenetic and therapeutic aspects]

Abstract

Familial Mediterranean fever was diagnosed in a 34-year-old Turkish patient with severe nephrotic oedema. Immunohistochemical classification of a biopsy specimen showed amyloidosis of the AA-type. There was a definite increase of serum amyloid-A-protein (SAA). The typical recurrent fever, attacks of abdominal pain with symptoms of subileus and joint swelling could be treated successfully with colchicine, the oedema with diuretics. The progression of renal failure and proteinuria as indicator of the degree of amyloid-induced renal damage remained unaffected by this treatment. With dimethyl-sulfoxide (DMSO) a marked improvement in renal function and a lowering of the SAA level could be achieved. Thus this treatment inhibits the progression of amyloidosis of the AA-type in Mediterranean fever and may be considered for other forms of AA-type amyloidoses. It is possible that the lowering of the SAA-serum concentration and the improvement of renal function is due to an antiphlogistic effect of DMSO, the mechanism of action of which is so far unknown.