Hippokratia 2007; 11 (4): 210-215
P. Boura, K. Tselios, P. Skendros, S. Kamali, A. Sarantopoulos, M. Raptopoulou-Gigi
Objective - Methods: Adamantiades-Behcet disease (ABD) is a multi-systemic vasculitis of unknown origin, with a characteristic geographic distribution, that affects vessels of all kinds and sizes and is characterized by recurrent mucosal, skin and ocular lesions. In the present study, a series of 36 patients from Northern Greece is analyzed retrospectively in regard to the epidemiological, clinical and immunological parameters. Results: All patients had recurrent oral ulcerations (36/36, 100%), while 23/36 (63.9%) experienced genital ulcerations
and 22/36 (61.1%) developed ocular disease. Skin manifestations were observed in 23/36 patients (63.9%) and pathergy test was found positive in 14/36 patients (38.9%). Other manifestations included central nervous system involvement, recurrent genitourinary inflammations, arthralgias and superficial thrombophlebitis. Laboratory findings were not specific, partly reflecting the severity of inflammation. Ocular disease was more often observed in HLA-B51 (+) patients (20/31, 64.5%) than in HLA-B51 (-) patients. Standard of care (SOC) treatment consisted of cyclosporine A, azathioprine, methylprednisolone and aspirin, whereas refractory disease was treated with intravenous pulses of methylprednisolone and cyclophosphamide. Occasionally, anti-TNF agents (infliximab) were applied to treat refractory ocular disease.
Conclusion: The findings of the present study come in agreement with those reported for other Mediterranean series. HLA-B51 seems to predispose to more severe disease, while early therapeutic intervention is beneficial for these patients.
Keywords: Adamantiades-Behcet disease, clinical manifestations, pathergy, HLA-B51