We report the case of a young pregnant patient with bilateral, sight-threatening retinal ischemic vasculopathy and systemic signs of Behçet disease. Although the systemic signs were highly suggestive of Behçet disease, the ocular presentation was unusual for this multisystemic inflammatory disorder. The differential diagnosis included a number of causes of ischemic retinal vasculopathy, such as systemic vasculitis, antiphospholipid syndrome, Susac syndrome, Purtscher-like retinopathy, and a new variant of acute macular neuroretinopathy (paracentral, acute middle maculopathy), which are further discussed.

Both IgG and IgM anti-beta2GPI assays were useful when clinical features of APS presented, even its standardization is ongoing. A decreased by half sensitivity of LA in PM compared with that in VT underlines the importance of adding anti-beta2GPI in PM of APS, especially IgM isotype although recent review questioned its significance.