Scleritis Definition

Diffuse or localized inflammation of the sclera. Scleritis is classified according to location:

❖ Anterior (inflammation anterior to the equator of the globe).

❖ Posterior (inflammation posterior to the equator of the globe). Anterior scleritis is further classified according to its nature:

❖ Non-necrotizing anterior scleritis (nodular or diffuse).

❖ Necrotizing anterior scleritis (with or without inflammation).

Epidemiology: Scleritis is far less frequent than episcleritis. Patients are generally older, and women are affected more often than men.

Etiology: Approximately 50% of scleritis cases (which tend to have severe clinical courses) are attributable to systemic autoimmune or rheumatic disease (Table 6.1), or are the result of immunologic processes associated with infection. This applies especially to anterior scleritis. Posterior scleritis is not usually associated with any specific disorder. As with episcleritis, scleritis is only occasionally due to bacterial or viral inflammation.

Symptoms and findings: All forms except for scleromalacia perforans are associated with severe pain and general reddening of the eye.

Anterior non-necrotizing scleritis (nodular form). The nodules consist of edematous swollen sclera and are not mobile (in contrast to episcleritis).

Table 6.1 Systemic diseases that can cause scleritis

Frequent causes

Rare causes

Rheumatoid arthritis

Polymyositis

Dermatomyositis

Ankylosing spondylitis

Spondylarthritis

Vasculitis

Wegener's granulomatosis Herpes zoster ophthalmicus Syphilis Gout

Tuberculosis Lues

Borreliosis Reiter's syndrome

— Diffuse non-necrotizing scleritis.

— Diffuse non-necrotizing scleritis.

Scleral Edema Treatment
Fig. 6.3 Typical signs include thickening and edema of the sclera and deep, diffuse reddening.

Anterior necrotizing scleritis (diffuse form). The inflammation is more severe than in the nodular form. It can be limited to a certain segment or may include the entire anterior sclera (Fig. 6.3).

Anterior necrotizing scleritis with inflammation. Circumscribed reddening of the eyes is a typical sign. There may be deviation or injection of the blood vessels of the affected region, accompanied by avascular patches in the episcleral tissue. As the disorder progresses, the sclera thins as the scleral lamellae of collagen fibrils melt, so that the underlying choroid shows through (Fig. 6.4). The inflammation gradually spreads from its primary focus. Usually it is associated with uveitis.

Anterior necrotizing scleritis without inflammation (scleromalacia per-forans). This form of scleritis typically occurs in female patients with a long history of seropositive rheumatoid arthritis. The clinical course of the disorder is usually asymptomatic and begins with a yellow necrotic patch on the sclera. As the disorder progresses, the sclera also thins so that the underlying choroid shows through. This is the only form of scleritis that may be painless.

Posterior scleritis. Sometimes there will be no abnormal findings in the anterior eye, and pain will be the only symptom. Associated inflammation of the orbit may result in proptosis (exophthalmos) and impaired ocular motility due to myositis of the ocular muscles. Intraocular findings may include exudative retinal detachment and/or choroid detachment. Macular and optic disk edema are frequently present.

Circumscribed scleral staphyloma secondary to scleritis. -

Fig. 6.4 The underlying choroid shows through at the bulge where the sclera is thinned, giving it a bluish tinge.

Circumscribed scleral staphyloma secondary to scleritis. -

H The reddening in scleritis is due to injection of the deeper vascular plexus on the sclera and to injection of the episclera. Inspecting the eye in daylight will best reveal the layer of maximum injection.

Differential diagnosis: Conjunctivitis and episcleritis (see that section).

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