Presumed autoimmune corneal endotheliopathy.

Two men (58 and 19 years of age) had an unusual recurrent, bilaterally symmetric disease process involving the cornea. It is characterized by stromal edema progressing centrally from the periphery in otherwise normal eyes. The corneal edema in each instance was closely associated with slowly moving linear keratic precipitates accompanied by the destruction of the endothelium, with minimal anterior chamber reaction. There was no history of herpetic keratitis or trauma, and serologic tests for syphilis were negative in both cases. A similar pattern of linear endothelial destruction has been reported heretofore only in association with corneal allograft rejection. This clinical pattern, the cytologic findings for the aqueous humor (macrophages and lymphocytes), and the rapid response to corticosteroid therapy suggested that an autoimmune process was the underlying cause of this disease.