Clinical Features and Management of Behçet Syndrome

Core Clinical Features

The European League Against Rheumatism recommends that Behçet syndrome diagnosis is based on recurrent oral aphthous ulcers plus at least two of the following: recurrent genital ulceration, uveitis/retinal vasculitis, specific skin lesions, or positive pathergy test 2, 4
Mucocutaneous manifestations, including recurrent oral aphthous ulcers, genital ulcers, and skin lesions, impair quality of life but do not cause permanent organ damage 1, 3
Ocular involvement, including posterior uveitis and retinal vasculitis, can lead to severe visual impairment or blindness if untreated 2
Vascular involvement affects approximately one-third of patients, involving both venous thrombosis and arterial disease, including aneurysms 2, 4
Gastrointestinal involvement can cause serious complications, including intestinal ulcers 1, 3

The American College of Rheumatology states that Behçet syndrome follows a chronic relapsing-remitting course with unpredictable exacerbations 1, 3
Disease manifestations typically ameliorate over time in many patients, allowing for treatment tapering or discontinuation 1, 3
Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis and can cause serious damage or death if untreated 1, 3, 4

A thorough genital examination is necessary to search for genital ulcers or scars, as recommended by the European League Against Rheumatism 2
Complete ophthalmologic examination is mandatory to assess for uveitis or retinal vasculitis, according to the American Academy of Ophthalmology 2
Inflammatory markers, such as ESR and CRP, can monitor disease activity but are not specific 2

When only skin, mucosa, and joint involvement exists, treatment can be tailored according to quality of life impact versus medication risks, as suggested by the European League Against Rheumatism 3
When major organ involvement occurs, rapid suppression of inflammation with immunosuppressives is critical to prevent irreversible loss of function, as recommended by the American College of Rheumatology 1, 3