Scleritis
Scleritis
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Scleritis is a severe, destructive, chronic, painful, and potentially blinding inflammatory disease of the Conjunctiva, Sclera, and Episclera tissues, the tough, white tissue that covers about four-fifths of the outer eye. The front fifth, the clear Cornea, is where light passes through the eye, enabling us to see.
Symptoms
Redness and severe eye pain, which may radiate to adjacent areas, the forehead, cheek, or behind the eye. This is usually associated with light sensitivity, teary, and in some cases, reduced or poor vision. The affected eye often has a bluish hue or becomes an intense purple.
There are several different sub-types of Scleritis: Nodular, Diffuse, Necrotizing Scleritis with inflammation, without inflammation (Scleromalacia Perforans), and Posterior Scleritis.
Nodular Scleritis
Nodular Scleritis is characterized by a focal area of inflammation, immovable, and tender, inflamed nodules on the eye.
Diffuse Scleritis
Diffuse Anterior Scleritis is the most common type, and is characterized by widespread inflammation of the Anterior portion of the Sclera, the white of the eye. The Diffuse type of Scleritis is, fortunately, the most benign form of Scleritis and the most responsive to therapy.
Necrotizing Scleritis
Necrotizing Scleritis is likely the worst form of the disease, sometimes leading to loss of the eye from multiple complications, severe pain, or occasionally perforation of the globe. It is often associated with severe systemic disease and involvement of multiple organs. An associated type of vascular inflammation, called Vasculitis, may threaten the lives of those patients afflicted. Pain with this condition is usually extreme, and damage to the Sclera is often marked.
Necrotizing scleritis also known as Scleromalacia Perforans is characterized by severe thinning of the Sclera of the Eye, allowing for local outpouchings of the underlying dark Uveal tissue. There are large abnormal blood vessels crossing areas of Scleral loss. The condition occurs in an otherwise white and "quiet" Eye, without pain. This type of Scleritis is associated with severe Rheumatoid Arthritis, occasionally seen in Wegener's Granulomatosis and Relapsing Polychondritis..
There are 2 types of Necrotizing Scleritis; with inflammation, without inflammation.
Posterior Scleritis
Posterior Scleritis is quite rare, but usually presents with poor or double vision, severe pain, proptosis (forward displacement of the eye), Uveitis (inflammation inside the Uvea Tract), and limitation of eye movement. An exudative Retinal detachment (fluid under the Retina) may cause severe visual loss, Angle-Closure Glaucoma from Choroidal effusion.
Cause
About 50% of Scleritis patients are associated with systemic autoimmune disorders, including Rheumatoid Arthritis, Gout, Wegener Granulomatosis, Relapsing Polychondritis, Systemic Lupus Erythematosus, Polyarteritis Nodosa, Ankylosing Spondylitis, with infections, or chemical or physical injuries. Sometimes the cause is unknown. It occurs most often in people between the ages of 30 and 60 (it is rare in children). Scleritis may be the initial or only presenting clinical manifestation of these potentially lethal disorders. The correct and rapid diagnosis and the appropriate systemic therapy can halt the relentless progression of both ocular and systemic processes, thus preventing destruction (perforation of the eyeball) and preventing blindness. Scary stuff!
Treatment
Scleritis is usually treated with both a topical eye solution and an oral medication containing corticosteroids. Nonsteroidal anti-inflammatories such as ibuprofen may be used for pain relief. Treatment systemically, that is, the underlying disorder must be controlled in order to halt the progression of the eye disease. This usually requires systemic steroids (e.g., prednisone) during acute stages, and immunosuppressive agents are often required for long-term control. Topical (eye-drop) steroids are not effective, although they are sometimes used to help distinguish between scleritis and episcleritis (episcleritis may be responsive to topical steroids). Steroid medications are not injected adjacent to the eye (e.g., subconjunctival steroid injections) as this may even lead to further Scleral thinning and perforation of the eye. If infection is the cause, your treatment will include antimicrobial medications. In severe cases, surgery may be required to graft injured areas of the eyeball.
Prognosis
Scleritis usually responds to treatment, but the condition may recur. If left untreated, Scleritis can lead to perforation of the eyeball. The long-term prognosis largely depends on what initially caused the condition. Ocular complications are common and may include Keratitis (inflammation of the Cornea), Cataracts (scarring of the Lens), Uveitis (inflammation of the Uvea Tract), and Glaucoma (elevated pressure in the eye that may lead to vision loss).
Links to References,
Ocular, and Immunologicial-Vasculitis Help
St Lukes Eye
EMedicine
Merck
HEALTHCENTRAL
Handbook of Ocular Disease
EyeMDLink
MEDLINEplus
Cleveland Clinic Immunologic Disease Center
Cole Eye Institute
John Hopkins Vaculitis Center
John Hopkins
Wilmer Eye Institute
National Eye Institute
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