Adamantiades-Behçet’s Disease

Abstract

Adamantiades-Behçet disease (ABD) is a distinct, chronic, relapsing systemic inflammatory disorder. ABD is not the typical autoinflammatory disease and should be viewed as a condition linking autoinflammation and autoimmunity classified among the primary systemic vasculitides. ABD exists worldwide, but it is more prevalent across the ancient trading route known as the “Silk Road”, i.e. in countries of the Mediterranean Basin, the Middle East and the Far East. The clinical spectrum is diverse, with significant differences in the prevalence of clinical manifestations between ethnic groups. Diagnosis is entirely clinical and in the absence of other explanations is based on the presence of recurring oral ulcerations (aphthous or herpetiform) at least three times in a 12-month period, plus at least any two of the following: recurrent genital aphthous ulceration or scarring, inflammation of the anterior and/or the posterior eye pole, skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions or acneiform nodules in postadolescent patients not on corticosteroids) and a positive pathergy test (non-specific skin hyperreactivity in response to minor trauma). Intestinal, joint and central nervous system involvement and vascular thrombosis and formation of arterial aneurysms also frequently occur. Morbidity and mortality are significant; ocular involvement results in blindness if left untreated in more than 70% of those affected. Treatment should be individualized balancing the risks of therapy with the putative efficacy of a given approach. For self-limited manifestations, the aim is to control symptoms. The prompt use of immunosuppressive agents, including anti-TNF monoclonal antibodies, is mandatory for vital organ involvement.