Serological and molecular HLA typing in Italian Behçet's patients: significant association to B51-DR5-DQw3 haplotype.
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Biomedical subjects
Publications and source records attributed to P Pivetti-Pezzi.
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The pathogenesis of vascular damage in Behçet's disease (BD) is still unknown. We investigated anticardiolipin antibodies (AC) in a genetically homogeneous group of Italian patients with BD and retinal vasculitis. In a subset, we examined the level of factor VIII-related antigen and antiendothelial cell antibodies (AECA). We found a high prevalence of AC; AECA were not found in most of our patients. Only three patients had raised levels of factor VIII-related antigen, all of whom had systemic involvement. We believe BD may be considered as a vasculitis of unknown origin in which several and different pathogenetic factors may play a role.
The efficacy, tolerability and safety of low doses (5-7 mg/kg/day) of oral ciclosporin (CS) for treatment of endogenous uveitis resistant to conventional therapy were studied in an open, noncontrolled, multicenter long-term trial. CS was shown to be effective for patients with posterior or intermediate noninfectious uveitis; it limited the progression of the disease, decreased the number and severity of inflammatory relapses, and improved visual acuity while reducing signs of intraocular inflammation. Moreover, in corticodependent patients, a steroid-sparing effect was also obtained. At low doses and by means of the careful monitoring of safety parameters, the side effects (especially renal function impairment) were well controlled. Efficacy was maintained during long-term treatment.
The prognostic value of ocular manifestations and their correlation with immune changes in HIV-infected subjects (75 PGL, 23 ARC, and 17 AIDS) have been longitudinally studied with an average follow-up of one year (3 to 22 months). The most common ocular manifestations were retinal cotton-wool-like spots, observed in 58.8% of AIDS patients and in 76.9% of those with ocular involvement. Two of three ARC patients who showed cotton-wool-like spots developed PCP a few weeks after ophthalmoscopic examination. A close correlation between ocular changes and decrease of CD4+ lymphocytes was observed. In our opinion, these ocular manifestations are as useful an indicator as opportunistic infections or AIDS-related neoplasias in the prognosis of HIV infection.
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Thirty-eight Italian patients with Behcet's disease, all with ocular involvement, (28 complete type and ten incomplete) were typed for HLA A,B,DR, and DQ antigens. A significant increase of HLA-B51 (p less than 0.00001) and DRw52 (p = 0.045) with no significant difference between complete and incomplete syndrome was found. The involvement of B51 antigen as the main immunogenetic factor in the disease is suggested by the high value of relative risk (RR = 16.03). However, the association with the II class antigen DRw52 (RR = 2.77) cannot be easily explained as a secondary association due to linkage disequilibria with B51.
Several immunological abnormalities have been described in Behçet's syndrome, the multisystem disease characterized by the triad of relapsing iridocyclitis with recurrent oral and genital ulcerations. In the present study we have evaluated T cell subsets in the peripheral blood of patients suffering from Behçet's syndrome, using a panel of anti-T cell monoclonal antibodies. When compared with normal subjects, patients with Behçet's syndrome show a discrepancy between the number of T3+ cells and those forming rosettes with sheep erythrocytes, a significantly higher number of T4+ and T8+ double labelled cells, as well as of T6+ lymphocytes in the peripheral blood. The percentage of T8 T lymphocytes is increased, thus lowering the T4+/T8+ cell ratio. The results of this study indicate that a complex imbalance of T cell subsets is present in Behçet's syndrome and give a rationale for possible treatment of these patients with immunomodulators.
Thirty patients with herpetic keratitis were allocated to a double-blind trial with either local treatment plus placebo (control group) or local treatment plus thymostimulin (TS group). The follow-up at 24 months demonstrated a significant reduction of recurrence rate among patients receiving thymostimulin, along with a significant increase of sheep rosette-forming cells (E rosette). Furthermore, among patients with superficial keratitis, thymostimulin treatment resulted in a significantly quicker corneal re-epithelization than placebo. Thymostimulin seems to be a safe and helpful drug in the management of herpetic keratitis.
The authors describe the results obtained at the Eye Department of Rome University using immunosuppressive drugs in the treatment of Behcet's disease. Of 31 patients with the disease, 23 were treated with different immunosuppressive drugs. The results obtained suggest that chlorambucil is the best therapeutic agent in this severe disease. As compared with other immunosuppressive agents used, such as azathioprine and ALGG, chlorambucil was the least toxic, the best tolerated and the most effective one.
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