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- CSS, Churg-Strauss syndrome
- PUK, peripheral ulcerative keratitis
- SLE, systemic lupus erythematosus
- WG, Wegener's granulomatosis
Eye complications are common in vasculitis, especially in Wegener's granulomatosis (WG), where up to 50% of all cases have ophthalmic involvement. Eye involvement in the Churg-Strauss syndrome (CSS) has been reported but is rare. Corneal autoantigens have been documented in patients with WG and CSS; in particular, circulating autoantibodies to corneal specific keratin 3 (K3) are present in 60% of patients with WG.1 Most of these patients have peripheral ulcerative keratitis (PUK), but others have no detectable eye disease. Antibodies to a second corneal antigen, BCEA-B, are associated with CSS.2
The eye is an immune privileged organ characterised by a reduced or modified immune response which protects the eye from inflammation and autoimmunity. Fas, together with its ligand FasL, has an important role in maintaining ocular immune privilege. Fas+ lymphoid cells that enter the eye are killed by apoptosis when the Fas binds …