Ocular Adnexal IgG4-Related Lymphoplasmacytic Infiltrative Disorder

Abstract

Ocular adnexal inflammation includes a wide spectrum of diseases, including idiopathic sclerosing inflammation and sclerosing dacryoadenitis. The pathogenesis of ocular adnexal sclerosing inflammation remains undetermined. Thus, these diseases have been considered a distinct clinical entity with histological characteristics similar to retroperitoneal fibrosis¹ or an idiopathic orbital inflammation that has progressed from the acute or subacute stage to a chronic inflammatory stage with fibrosis.^2,3 Some cases of ocular adnexal sclerosing inflammation may progress to ocular adnexal lesions with multifocal fibrosclerosis.^2,4,5