Progressive subretinal fibrosis and blindness in patients with multifocal granulomatous chorioretinitis.

Abstract

To describe the clinical and histopathologic findings in four eyes of three patients who became blind because of multifocal choroiditis and massive subretinal fibrosis. Clinicopathologic correlative study. During a period of several years, three healthy elderly patients developed severe visual loss only partly explained by multifocal chorioretinitis and massive subretinal fibrosis. Histopathologic examination of four eyes from these patients disclosed widespread destruction of the outer retina and retinal pigment epithelium, massive areas of subretinal fibrous tissue proliferation, granulomatous inflammation centered around degenerated and fragmented Bruch's membrane, and chronic uveitis. No infectious organisms were identified by special stains or electron microscopy in one eye. Clinical and histopathologic findings in these three patients were consistent with an autoimmune disease process directed at the retina, retinal pigment epithelium, inner choroid, or all three. Cellular injury in this location can result in massive subretinal fibrosis. Subretinal fibroplasia, however, is probably a nonspecific reparative response to injury. The pathogenesis of this blinding disorder in elderly patients may be similar to the less severe disease usually occurring in younger patients with multifocal choroiditis, panuveitis, and punctate inner choroiditis.