Therapeutic alternatives in Behçet's syndrome

Behçet's Syndrome (BD) is a chronic, relapsing, recurrent systemic vasculitis with an unknown cause. The disease affects all organs of the body concurrently or consecutively. Its various clinical manifestations result from ubiquitous small-vessel vasculitis, which is the underlying pathology. An Italian study has reported an increased association of the extended haplotype B51-DR5-DQw3. Without a known etiology BD syndrome has no uniformly acceptable therapy. Our study addresses therapeutic alternatives for the treatment of BD, with the systemic use of interferon alpha-2a., which has antiviral. immunomodulatory, antiproliferative, and antitumoral properties. Ten patients diagnosed with BD were referred from September 2002 to September 2005 to the Department of Gynaecology, Obstetrics and Reproduction of the Second University of Naples. The International Study Group (ISG) Criteria for Behçet's Disease (27) was applied. Patients were treated with oral prednisone; sulfasalazine; clobetasol; and interferon alpha-2a. Every month all patients had a complete blood count, platelet count, and liver function test. Biopsies of genital ulcerations identified small vessel vasculitis with mononuclear cell and lynphocytic infiltrates. HLA-B27 and B5 were positive in three subjects. The pathergy test was positive in all patients. Today the therapy is still ongoing, and none of the patients in therapy with our protocol present clinical symptoms of BD or intolerance. Laboratory findings are in a normal range and none have had neurological failure. Our findings may be attributable to less severe disease in a patients, to our smaller number of patients, or to other unknown factors. Nonetheless, these findings remain to be confirmed in a larger number of patients.