Diagnosis

The diagnosis of Adamantiades-Behget's is a clinical one, as there are not yet any specific biomarkers for disease available. Several sets of diagnostic criteria are available to assist in making the diagnosis of Adamantiades-Behget's, each with varying sensitivity and specificity for disease classification. Perhaps the most commonly used set of criteria is the International Study Group's Criteria for the Diagnosis of Adamantiades-Behget's Disease, established in 1990 (Table 2) (30). This set of criteria has a sensitivity of 91% and specificity of 96%, although some feel that its inclusion of acneiform lesions decreases specificity, and recommend that these lesions, if present, be biopsied to confirm vasculitis (4).

Nonspecific tests including inflammatory anemia and markers such as elevated erythrocyte sedimentation rate and C-reactive protein can be present during flares. Serum-

soluble ICAM-1, a marker of neutrophil activation, has been shown to be elevated in patients with active disease; however, its use in diagnosis is limited (31).

Antibodies against the endothelial cell component a-enolase may prove to be specific markers for disease activity, but further studies are needed (9,10). Antinuclear antibodies, antineutrophil cytoplasmic antibodies, and rheumatoid factor are not associated with disease, and, if present, may indicate that another autoimmune process is present. Testing for the Adamantiades-Behget's-associated HLA-B51 or -B52 alleles is not commonly performed for diagnostic purposes.

Further evaluation for involvement of specific organ systems should be performed as symptoms and signs dictate, although due to potentially catastrophic consequences if lung aneurysms are missed, some advocate pulmonary imaging in all cases of Adamantiades-Behcet's (16).

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