[Familial Mediterranean fever (paroxismal polyserositis, familial recurring polyserositis, periodic disease)].

[familial mediterranean fever]

Paroxismal polyserositis is an orphan disease most often affecting Mediterranean populations . It is caused by a mutation on chromosome 16 leading to pyrine synthesis disorder . The disease has a characteristic clinical picture , the most prominent manifestation being recurrent aseptic inflammation of serous membranes especially in peritoneum , marked temperature reaction , and apparent spontaneous recovery in the attack-free period . Inadequate or excessively intense treatment may cause complication in the form of secondary amyloidosis of internal organs . The most effective therapeutic modality is daily intake of colchicine at a dose of 1 -1 .5 mg .