T cell mediated immune responses against the cornea specific
protein BCP 54 have been observed in patients with
uveitis, Fuchs'
heterochromic cyclitis, and
corneal disease. The pathophysiological role of this anti-
BCP 54 response in
corneal disease is not known. In order to ascertain whether the presence of such an immune response is related to the
corneal disease itself or related to genetic influences, the anti-
BCP 54 response was determined in 104 patients with severe
corneal disease, using a monocyte migration inhibition assay. The results were compared with the presence of a variety of ocular parameters as well as with the distribution of
HLA antigens in these patients. While only 7% of healthy controls responded to
BCP 54, 37% of the patients showed a positive response (p = 0.002); in particular, patients with previous graft rejection, non-
herpetic keratitis, and bullous keratopathy reacted against
BCP 54. No relation with known risk factors for
corneal transplantation, such as corneal neovascularisation, was observed. No significant association with the presence of any of the
HLA antigens was observed. It was concluded that the main inducer of an anti-
BCP 54 response is
corneal disease itself, and that the presence of
corneal disease is able to break the immunological privilege typical of normal corneas.