between surgery and the onset of scleritis varied from 2 weeks to 24 months. Four patients (57%) were found to have an infectious process. In another study performed by us [161], 91% of the patients were found to have an underlying autoimmune vasculitic systemic disease. Immune com- plex-mediated vasculitis and T-cell-mediated immune responses can be triggered in patients with underlying systemic autoimmune vasculitic or infectious diseases after ocular surgical trauma resulting in necrotizing scleritis. These Þndings emphasize the need for meticulous diagnostic pursuit of systemic autoimmune vasculitic or infectious diseases in patients with necrotizing scleritis following intraocular surgery.

4.3Summary

Episcleritis is a benign disease that occurs in young adults, usually women, with a peak incidence in the fourth decade. Although the course is self-limiting, episcleritis is usually characterized by recurrences involving both eyes at the same time or different eyes at different times. It presents as an uncomfortable red eye, sometimes with tearing and mild photophobia. Pain, if any, is mild and localized to the eye. Clinical examination with a slit lamp discloses that the inßammation is entirely localized within the episcleral tissue, never involving the underlying sclera. Redness diminishes greatly after instillation of topical phenylephrine (10%) since this vasoconstrictor has great effect on the superÞcial episcleral vessels, which are congested in episcleritis. Episcleritis rarely causes decrease in vision, corneal involvement, uveitis, or glaucoma. Two types of episcleritis may be distinguished: simple and nodular. In simple episcleritis, in which there is a diffuse vascular congestion, the course lasts between 5 and 10 days without treatment. In nodular episcleritis, in which one or more nodules form, the course lasts between 4 and 6 weeks without treatment. Approximately 27% of patients have an underlying associated disease, including 15% with connective tissue diseases, 6% with infectious diseases, 5% with rosacea, and 1% with atopy.

Unlike episcleritis, scleritis is a severe inßammatory disease that can be progressively destructive, sometimes leading to loss of vision or loss of the eye. Furthermore, scleritis may be the presenting manifestation of a potentially lethal systemic vasculitic disease or may herald the onset of an occult systemic vasculitis in a patient with an already diagnosed systemic disease that is apparently in remission. Scleritis is most common in the fourth to sixth decades of life, more often in women, with a peak incidence in the Þfth decade. Scleritis is also characterized by recurrences involving both eyes at the same time or different eyes at different times. It presents with deep severe pain, often radiating to the forehead, the jaw, and the sinuses. Other symptoms include tearing and mild photophobia. The main sign is redness that, unlike episcleritis, has a bluish tinge, best seen under natural light. Clinical examination with a slit lamp discloses that the inßammation is localized within the episcleral and scleral tissue. Redness does not diminish after instillation of topical phenylephrine (10%) because this vasoconstrictor has minimal effect on the deep episcleral vessels, which are congested in scleritis. Scleritis may cause decrease in vision, corneal involvement, uveitis, glaucoma, cataract, exudative retinal detachment, and macular edema. Two types of sclerits may be distinguished: anterior and posterior. Anterior scleritis may be further classiÞed into diffuse, nodular, necrotizing with inßammation (necrotizing), and necrotizing without inßammation (scleromalacia perforans). Necrotizing scleritis is the most severe and destructive form because it may lead to the loss of the eye from multiple complications and because it is highly associated with potentially lethal systemic vasculitic diseases. Approximately 36% of the patients have an underlying associated disease, including 25% with connective tissue or vasculitic diseases, 10% with infectious diseases, and 1% with miscellaneous diseases (rosacea, foreign body). It is, therefore, extremely important that the correct diagnosis is made and that subsequent adequate treatment is given as early as possible during the course of the disease.

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