Scleritis

Scleritis: Inflammation limited to the sclera. Note markedly dilated blood vessels which do not extend onto the underside of the lower lid, helping to distinguish scleritis from conjunctivitis.
(Image courtesy of Charlie Goldberg, M.D.)
ICD-10 | H15.0
ICD-9 | 379.0
DiseasesDB | 11898
MedlinePlus | 001003 scleritis. MedlinePlus2
eMedicine | emerg/521 oph/642

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Rohan Bir Singh, M.B.B.S.[2]

## Scleral Anatomy

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The word "sclera" is derived from Greek word, "sklēros" meaning hard. It consists of the posterior five-sixths part of the fibrous outer tunic of the eye. The outer scleral surface is in contact with Tenon’s capsule and the bulbar conjunctiva. The sclera is covered by a thin layer of loose tissue called episclera. It is separated from the choroid by the suprachoroidal space. The extraocular muscles are also inserted in the sclera. The thickness of the sclera varies from place to place. The thickest part is at the posterior pole and the thinnest underneath the insertion of rectus muscles.

At the point of entrance for the optic nerve in the orbit, the sclera is consists of a sieve-like membrane, the lamina cribrosa, which allows the passage of fasciculi of the nerve. The sclera receives the circulation of two long and ten to twelve short posterior ciliary arteries around the optic nerve. Slightly posterior to the equator, four vortex veins (venae vorticosae) drain the sclera. The anterior ciliary arteries and veins penetrate the sclera nearly 3 to 4 mm away from the limbus.

The histology of the sclera consists of three layers - episcleral tissue, sclera proper and lamina fusca. The episcleral tissue comprises fine loose elastic tissue fibers and contains a large number of small vessels. The sclera proper is formed by dense bands of parallel and interlacing collagen fibers. The collagen fiber bundles are arranged in concentric circles at the limbus and around the entrance of the optic nerve, elsewhere the arrangement is quite complicated. The lamina fusca has a brown color owing to the presence of a large number of branched chromatophores. The sclera is almost avascular and its histological structure resembles that of the cornea. However, sclera is opaque due to the hydration and irregular arrangement of its lamellae. The nerve supply of sclera comes through the ciliary nerves.

## Scleritis

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Scleritis is a serious inflammatory disease that affects the sclera. The disease is often contracted through association with other diseases of the body, such as Wegener's granulomatosis or rheumatoid arthritis; it can also be attained through disorders of menstruation. For this reason, scleritis occurs frequently among young women. There are three types of scleritis: diffuse scleritis (the most common), nodular scleritis, and necrotizing scleritis (the most severe). Scleritis may be the first symptom of onset connective tissue disease[3].

The term "Episcleritis" refers to inflammation of the episclera.

## Classification

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Inflammation of the sclera is classified by its location and severity using the system devised by Watson.[1]

Classification of Scleritis

Episcleritis  |

* Diffuse
* Nodular

Scleritis  |

* Scleritis – anterior

1. Diffuse
2. Nodular
3. Necrotizing
* with inflammation
* without inflammation
4. scleromalacia perforans

* Scleritis – posterior

1. Diffuse
2. nodular
3. necrotizing

## Pathophysiology

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AUTOIMMUNE DISEASE Systemic autoimmune disease is a common finding in patients with scleritis,2 and scleral and episcleral tissues appear to be particularly at risk in a wide range of autoimmune disorders. Why this should be the case is not clear. It is possible that everyday exposure to irritants and pathogens provokes minor episodes of inflammation that in some way prime the autoimmune-mediated inflammation. The following description includes details of histology of some cases where precise information concerning aetiology was not provided, but it has been assumed that the cases were of autoimmune aetiology. The detailed pathogenesis of this heterogeneous group of conditions remains uncertain although it is agreed that autoimmunity plays an important role. Immune complex deposition may be a feature1–3 and, interestingly, one case of scleritis following treatment with a mouse monoclonal antibody has been reported.4 Immune complex formation has also been described in at least one pathological report.5 In conditions such as relapsing polychondritis, autoantibodies, in this instance to type II collagen, may have a role to play. It has been proposed that the presence of higher levels of complement C1 in the anterior sclera may explain the increased incidence of immune complex disease-associated scleritis at the front of the eye.6 Necrotizing and non-necrotizing vasculitis (especially arteritis) have been seen in some cases.2,7 The early involvement of sclera adjacent to anterior and posterior ciliary vessels has been used to support the notion that vasculitis is an important component in the pathogenesis of scleritis,8 but non-vasculitic inflammatory diseases, such as multiple sclerosis, are also characterized by perivascular pathology. In addition, in many cases vasculitis is not found and, although this may to a degree reflect problems with sampling, it seems probable that vasculitis is not a universal finding. Interestingly, other vascular changes, in the absence of inflammation, vasculitic or otherwise, have been reported. These include vascular closure,9 which might be anticipated to lead to ischaemic necrosis in the absence of vasculitis. There has been relatively little analysis of the detailed phenotype of the inflammatory cell infiltrate. In one study of two cases there was a predominance of CD4 positive T cells with clusters of B cells in a perivascular location.10 In this study no evidence of vasculitis was seen. Relatively few of the T cells appeared activated as

## Signs and symptoms

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Symptoms of the disease include:

* Redness of the sclera and conjunctiva, sometimes changing to a purple hue
* Severe ocular pain (not present in episcleritis) which may radiate to the temple or jaw
* Increased light sensitivity and tearing
* Decrease in visual acuity, possibly leading to blindness

## Diagnosis

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Scleritis is best detected by examining the sclera in daylight; retracting the lids helps determine the extent of involvement. Other aspects of the eye exam (i.e. visual acuity testing, slit lamp examination, etc) can be normal. Ancillary tests CT scans, MRIs, and ultrasonographies can be helpful, but do not replace the physical examination.

## Treatment

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In very severe cases of necrotizing scleritis, eye surgery must be performed to repair damaged corneal tissue in the eye and preserve the patient's vision. For less severe cases, nonsteroidal anti-inflammatory drugs, such as ibuprofen, are prescribed for pain relief. Scleritis itself is treated with an oral medication containing corticosteroids and an eye solution. In some cases, antibiotics are prescribed. Simply using eye drops will not treat scleritis. If not treated, scleritis can cause blindness.

## References

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1. ↑ Watson PG, Hayreh SS (1976). "Scleritis and episcleritis". Br J Ophthalmol. 60 (3): 163–91. PMC 1042706. PMID 1268179.