Familial Mediterranean Fever: a review for clinical management.

Familial Mediterranean Fever (FMF) is a hereditary autosomal recessive, autoinflammatory disorder characterized by recurrent, self-limiting episodes of short duration (mean 24-72 h) of fever and serositis. FMF is the most frequent periodic febrile syndrome among the autoinflammatory syndromes (AS), a heterogeneous group of recently identified diseases clinically characterized by recurrent febrile attacks, in the absence of autoantibodies and antigen-specific T lymphocytes. In FMF, periodic attacks show inter- and intra-individual variability in terms of frequency and severity. Usually, they are triggered by apparently innocuous stimuli and may be preceded by a prodromal period. The Mediterranean FeVer gene (MEFV) responsible gene maps on chromosome 16 (16p13) encoding the pyrin-marenostrin protein. The precise pathologic mechanism is still to be definitively elucidated; however a new macromolecular complex, called inflammasome, seems to play a major role in the control of inflammation and it might be involved in the pathogenesis of FMF. The most severe long-term complication is type AA amyloidosis, principally affecting the kidney and the cause of chronic renal failure. Two types of risk factors, genetic and non-genetic, have been identified for this complication. Currently, the only effective treatment of Familial Mediterranean Fever is the colchicine. New drugs in a few colchicine resistant patients have been tried, but additional studies on larger series are necessary to draw definitive conclusions.

Efficacy of etanercept in the treatment of a patient with Behçet's disease.

Behçet's disease is a systemic inflammatory disorder without a specific treatment that is chosen on the basis of the type and severity of manifestation in the organ involved. More recently, biological agents like etanercept have emerged as possible therapeutic alternatives in patients resistant to conventional therapy. We describe the successful treatment for 1 year of resistant Behçet's disease with etanercept. After the administration of this drug, a resolution of the clinical, laboratory and instrumental picture was achieved with a suspension of immunosuppressive treatments and a reduction of steroid dependency (5 mg/day). No side effects were observed.