A Young Woman With Unilateral Eye Pain and Erythema

Thalia Salinas; Rod Foroozan, MD


April 10, 2014


The sclera is the protective outer layer of the eye, made of connective tissue. It starts at the limbus, where it is continuous with the cornea, and terminates at the optic canal, where it fuses with the dura mater and arachnoid sheaths of the optic nerve. Sensory innervation comes from the short and long posterior ciliary nerves. Because the extraocular muscles insert into the sclera, the pain of scleritis may be worsened with eye movement. The sclera is avascular; thus, it depends on the vascular plexus within the conjunctiva and the 2 vascular layers of the episclera (superficial and deep). The superficial episcleral vessels are radially arranged, and the deep plexus closely overlies the sclera.

The term "scleritis" refers to inflammation of the sclera. It usually presents as a painful inflammatory process of the sclera that may also involve the cornea, episclera, and uvea, thus threatening vision. It affects women more commonly than men, and usually presents between 30 and 50 years of age.[2] The prevalence is about 6 cases per 100,000 population.[2]

In about 50% of patients, scleritis is a manifestation of an underlying systemic disease, such as rheumatoid arthritis, Wegener granulomatosis, relapsing polychondritis, inflammatory bowel disease, systemic lupus erythematosus, polyarteritis nodosa, and seronegative spondyloarthropathies. An associated infection is found in 4%-18% of patients, with herpes zoster being the most frequent.[3] Other causes, such as cancer, medications, and trauma, are rare.

Scleritis is classified as anterior or posterior, with anterior scleritis being further divided into diffuse, nodular, or necrotizing. Necrotizing scleritis is the most severe form. Ocular pain is typically the chief symptom in scleritis. It may be dull or aching, severe, or constant, and worsened with eye movement. With anterior scleritis, injection of the eye is usually observed, and posterior scleritis may lead to ptosis and periorbital tissue swelling. Other symptoms include tearing and photosensitivity. Ocular complications, such as anterior uveitis, peripheral keratitis, glaucoma, and posterior scleritis causing optic disc edema, may cause vision loss.[2]

In scleritis, the conjunctival and superficial episcleral vascular plexuses may be involved, but the distinguishing finding is involvement of the deep episcleral vessels. The deep episcleral vessels are dilatated and engorged, giving a characteristic violaceous color that may be better appreciated in natural light.[4] The deep episcleral vessels are displaced outward by the swollen sclera. The conjunctival and superficial episcleral vessels may blanch with phenylephrine, whereas the deep vessels are unaffected.

An evaluation for systemic causes should be considered, including blood pressure, renal function, and acute-phase reactants.[4] Investigation may include testing of antinuclear antibodies, anticytoplasmic antibodies, and rheumatoid factor. Further testing is determined by the patient's history and underlying conditions.

The first-line treatment in nonnecrotizing, noninfectious scleritis is a nonsteroidal anti-inflammatory drug (NSAID).[5] In more severe cases, such as posterior scleritis or necrotizing scleritis, or when NSAIDs are ineffective, oral corticosteroids and periocular injections may be used.[5] For prolonged treatment or in patients with contraindications to corticosteroids, steroid-sparing immunosuppressive drugs, such as methotrexate, cyclophosphamide, and mycophenolate mofetil, may be used. For refractory disease, tumor necrosis factor alpha antagonists, including infliximab and adalimumab, have been used.[5]


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