Scleritis

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Scleritis
Scleritis.png
Inflammation of entire thickness of the sclera
Specialty Ophthalmology, Optometry
Symptoms Eye redness, pain, photophobia, tearing, decrease in visual acuity
Usual onset30 - 60 years of age
Risk factors Female gender
Differential diagnosis Episcleritis
Treatment Non-steroidal anti-inflammatory drugs
Surgery (rarely needed)
FrequencyUncommon

Scleritis is a serious inflammatory disease that affects the white outer coating of the eye, known as the sclera. The disease is often contracted through association with other diseases of the body, such as granulomatosis with polyangiitis or rheumatoid arthritis. 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 of connective tissue disease. [1]

Contents

Episcleritis is inflammation of the episclera, a less serious condition that seldom develops into scleritis. [2]

Signs and symptoms

Scleral translucency following recurrent scleritis. Recurrent scleritis.jpg
Scleral translucency following recurrent scleritis.

Symptoms of scleritis include: [3]

The pain of episcleritis is less severe than in scleritis. [4] In hyperemia, there is a visible increase in the blood flow to the sclera (hyperaemia), which accounts for the redness of the eye. Unlike in conjunctivitis, this redness will not move with gentle pressure to the conjunctiva.

Complications

Secondary keratitis or uveitis may occur with scleritis. [4] The most severe complications are associated with necrotizing scleritis.

Pathophysiology

Most of the time, scleritis is not caused by an infectious agent. [5] Histopathological changes are that of a chronic granulomatous disorder, characterized by fibrinoid necrosis, infiltration by polymorphonuclear cells, lymphocytes, plasma cells and macrophages. The granuloma is surrounded by multinucleated epitheloid giant cells and new vessels, some of which may show evidence of vasculitis.

Diagnosis

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.) may be normal. Scleritis may be differentiated from episcleritis by using phenylephrine or neosynephrine eye drops, which causes blanching of the blood vessels in episcleritis, but not in scleritis. [4]

Ancillary tests CT scans, MRIs, and ultrasonographies can be helpful, but do not replace the physical examination.

Classification

Scleritis can be classified as anterior scleritis and posterior scleritis. Anterior scleritis is the most common variety, accounting for about 98% of the cases. It is of two types : Non-necrotising and necrotising. Non-necrotising scleritis is the most common, and is further classified into diffuse and nodular type based on morphology. Necrotising scleritis accounts for 13% of the cases. It can occur with or without inflammation.

Treatment

Medical

In mild to moderate cases of scleritis, non-steroidal anti-inflammatory drugs such as flurbiprofen, indomethacin or ibuprofen may be prescribed for pain relief. [6] Systemic corticosteroids like prednisolone may be used if NSAIDs are inappropriate or scleritis is posterior or necrotizing. [7] Periocular steroid injections may be used in non-necrotizing scleritis, but it is contraindicated in necrotizing disease. [7] If disease control is inadequate with steroids alone, immunosuppressives (Eg: cyclophosphamide, azathioprine, methotrexate) and/or immunomodulators may be considered for treatment. [7] In infective scleritis, if infective agent is identified, topical or systemic antibiotics may be prescribed. [7] [8]

Surgical Intervention

Surgery may be indicated if scleral perforation or excessive scleral thinning is present. [6] Bandage contact lens or corneal glue may be used to repair damaged corneal tissue in the eye and preserve the patient's vision. [6] If not treated, scleritis can cause blindness.

Epidemiology

Scleritis is not a common disease, although the exact prevalence and incidence are unknown. It is somewhat more common in women, and is most common in the fourth to sixth decades of life. [9]

Related Research Articles

Conjunctivitis Inflammation of the eye

Conjunctivitis, also known as pink eye, is inflammation of the outermost layer of the white part of the eye and the inner surface of the eyelid. It makes the eye appear pink or reddish. Pain, burning, scratchiness, or itchiness may occur. The affected eye may have increased tears or be "stuck shut" in the morning. Swelling of the white part of the eye may also occur. Itching is more common in cases due to allergies. Conjunctivitis can affect one or both eyes.

Sclera White part of an eyeball

The sclera, also known as the white of the eye or, in older literature, as the tunica albuginea oculi, is the opaque, fibrous, protective, outer layer of the human eye containing mainly collagen and some crucial elastic fiber. In humans, and some other vertebrates, the whole sclera is white, contrasting with the coloured iris, but in most mammals, the visible part of the sclera matches the colour of the iris, so the white part does not normally show while other vertebrates have distinct colors for both of them. In the development of the embryo, the sclera is derived from the neural crest. In children, it is thinner and shows some of the underlying pigment, appearing slightly blue. In the elderly, fatty deposits on the sclera can make it appear slightly yellow. People with dark skin can have naturally darkened sclerae, the result of melanin pigmentation.

Cornea Transparent front layer of the eye

The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Along with the anterior chamber and lens, the cornea refracts light, accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is approximately 43 dioptres. The cornea can be reshaped by surgical procedures such as LASIK.

Eye surgery Surgery performed on the eye or its adnexa

Eye surgery, also known as ocular surgery, is surgery performed on the eye or its adnexa, typically by an ophthalmologist. The eye is a very fragile organ, and requires extreme care before, during, and after a surgical procedure to minimise or prevent further damage. An expert eye surgeon is responsible for selecting the appropriate surgical procedure for the patient, and for taking the necessary safety precautions. Mentions of eye surgery can be found in several ancient texts dating back as early as 1800 BC, with cataract treatment starting in the fifth century BC. Today it continues to be a widely practiced type of surgery, with various techniques having been developed for treating eye problems.

Dry eye syndrome Medical condition

Dry eye syndrome (DES), also known as keratoconjunctivitis sicca (KCS), is the condition of having dry eyes. Other associated symptoms include irritation, redness, discharge, and easily fatigued eyes. Blurred vision may also occur. Symptoms range from mild and occasional to severe and continuous. Scarring of the cornea may occur in untreated cases.

Uveitis Inflammation of the uvea of the eye

Uveitis is the inflammation of the uvea, the pigmented layer that lies between the inner retina and the outer fibrous layer composed of the sclera and cornea. The uvea consists of the middle layer of pigmented vascular structures of the eye and includes the iris, ciliary body, and choroid. Uveitis is described anatomically, by the part of the eye affected, as anterior, intermediate or posterior, from front to back. In the panuveitic form, all parts are involved. The commonest is the anterior form. Symptoms include pain, floaters and blurred vision, and clinical examination may show redness and an irregular pupil, while opthalmic examination shows dilated ciliary blood vessels and the presence of cells in the anterior chamber. Uveitis may arise spontaneously and associated with some genetic factors, or be associated with a wide range of conditions including autoimmune disease and infections. While the eye is a relatively protected environment, its immune mechanisms may be overcome resulting in inflammation and tissue destruction associated with T-cell activation. The incidence is approximately 1:4500, most commonly between the ages of 20–60.

Red eye (medicine) Eye that appears red due to illness / injury. It is a common diseases

A red eye is an eye that appears red due to illness or injury. It is usually injection and prominence of the superficial blood vessels of the conjunctiva, which may be caused by disorders of these or adjacent structures. Conjunctivitis and subconjunctival hemorrhage are two of the less serious but more common causes.

Allergic conjunctivitis Medical condition

Allergic conjunctivitis (AC) is inflammation of the conjunctiva due to allergy. Although allergens differ among patients, the most common cause is hay fever. Symptoms consist of redness, edema (swelling) of the conjunctiva, itching, and increased lacrimation. If this is combined with rhinitis, the condition is termed allergic rhinoconjunctivitis (ARC).

Fuchs dystrophy Medical condition

Fuchs dystrophy, also referred to as Fuchs endothelial corneal dystrophy (FECD) and Fuchs endothelial dystrophy (FED), is a slowly progressing corneal dystrophy that usually affects both eyes and is slightly more common in women than in men. Although early signs of Fuchs dystrophy are sometimes seen in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s.

Scleral lens Large contact lens resting on the sclera, creating a tear-filled vault over the cornea

A scleral lens, also known as a scleral contact lens, is a large contact lens that rests on the sclera and creates a tear-filled vault over the cornea. Scleral lenses are designed to treat a variety of eye conditions, many of which do not respond to other forms of treatment.

Granulomatous meningoencephalitis (GME) is an inflammatory disease of the central nervous system (CNS) of dogs and, rarely, cats. It is a form of meningoencephalitis. GME is likely second only to encephalitis caused by canine distemper virus as the most common cause of inflammatory disease of the canine CNS. The disease is more common in female dogs of young and middle age. It has a rapid onset. The lesions of GME exist mainly in the white matter of the cerebrum, brainstem, cerebellum, and spinal cord. The cause is only known to be noninfectious and is considered at this time to be idiopathic. Because lesions resemble those seen in allergic meningoencephalitis, GME is thought to have an immune-mediated cause, but it is also thought that the disease may be based on an abnormal response to an infectious agent. One study searched for viral DNA from canine herpesvirus, canine adenovirus, and canine parvovirus in brain tissue from dogs with GME, necrotizing meningoencephalitis, and necrotizing leukoencephalitis, but failed to find any.

Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was first described by D. G. Cogan in 1945.

Vernal keratoconjunctivitis Medical condition

Vernal keratoconjunctivitis (VKC) is a recurrent, bilateral, and self-limiting inflammation of conjunctiva, having a periodic seasonal incidence.

Episcleritis Medical condition

Episcleritis is a benign, self-limiting inflammatory disease affecting part of the eye called the episclera. The episclera is a thin layer of tissue that lies between the conjunctiva and the connective tissue layer that forms the white of the eye (sclera). Episcleritis is a common condition, and is characterized by the abrupt onset of painless eye redness.

Pinguecula Medical condition

A pinguecula is a common type of conjunctival stromal degeneration in the eye. It appear as an elevated yellow-white plaque in the bulbar conjunctiva near to limbus. Calcification may also seen occasionally.

Corneal ulcer Medical condition

Corneal ulcer is an inflammatory or, more seriously, infective condition of the cornea involving disruption of its epithelial layer with involvement of the corneal stroma. It is a common condition in humans particularly in the tropics and the agrarian societies. In developing countries, children afflicted by Vitamin A deficiency are at high risk for corneal ulcer and may become blind in both eyes, which may persist lifelong. In ophthalmology, a corneal ulcer usually refers to having an infectious cause while the term corneal abrasion refers more to physical abrasions.

Scleral tattooing

Scleral tattooing is the practice of tattooing the sclera, or white part of the human eye. The dye is not injected into the tissue, but between two layers of the eye, where it spreads out over a large area. The process is not common as there are few professionals comfortable doing it.

Herpes simplex keratitis Medical condition

Herpetic simplex keratitis is a form of keratitis caused by recurrent herpes simplex virus (HSV) infection in the cornea.

Sickle cell retinopathy can be defined as retinal changes due to blood vessel damage in the eye of a person with a background of sickle cell disease. It can likely progress to loss of vision in late stages due to vitreous hemorrhage or retinal detachment. Sickle cell disease is a structural red blood cell disorder leading to consequences in multiple systems. It is characterized by chronic red blood cell destruction, vascular injury, and tissue ischemia causing damage to the brain, eyes, heart, lungs, kidneys, spleen, and musculoskeletal system.

Peripheral Ulcerative Keratitis (PUK) is a group of destructive inflammatory diseases involving the peripheral cornea in human eyes. The symptoms of PUK include pain, redness of the eyeball, photophobia, and decreased vision accompanied by distinctive signs of crescent-shaped damage of the cornea. The causes of this disease are broad, ranging from injuries, contamination of contact lenses, to association with other systemic conditions. PUK is associated with different ocular and systemic diseases. Mooren's ulcer is a common form of PUK. The majority of PUK is mediated by local or systemic immunological processes, which can lead to inflammation and eventually tissue damage. Standard PUK diagnostic test involves reviewing the medical history and a completing physical examinations. Two major treatments are the use of medications such as corticosteroids or other immunosuppressive agents and surgical resection of the conjunctiva. The prognosis of PUK is unclear with one study providing potential complications. PUK is a rare condition with an estimated incidence of 3 per million annually.

References

  1. "Scleritis". WebMD, LLC. Medscape. Retrieved 27 November 2012.
  2. "Episcleritis: MedlinePlus Medical Encyclopedia". Bethesda, MD: United States National Library of Medicine . Retrieved 20 June 2010.
  3. Vorvick, Linda J. (July 28, 2010). "Scleritis". PubMed Health. United States National Library of Medicine . Retrieved July 6, 2011.
  4. 1 2 3 Goldman, Lee (2011). Goldman's Cecil Medicine (24th ed.). Philadelphia: Elsevier Saunders. p. 2440. ISBN   978-1437727883.
  5. Yanoff, Myron; Jay S. Duker (2008). Ophthalmology (3rd ed.). Edinburgh: Mosby. pp. 255–261. ISBN   978-0323057516.
  6. 1 2 3 "Scleritis". eyewiki.aao.org.
  7. 1 2 3 4 John F., Salmon (2020). "Episclera and sclera". Kanski's clinical ophthalmology : a systematic approach (9th ed.). Edinburgh: Elsevier. ISBN   978-0-7020-7713-5. OCLC   1131846767.
  8. Ramanjit, Sihota; Radhika, Tandon (2015). Parsons' diseases of the eye (22nd ed.). New Delhi, India: Elsevier. ISBN   978-81-312-3819-6. OCLC   905915528.
  9. Maite Sainz de la Maza (Feb 15, 2012). The sclera (2nd ed.). New York: Springer. p. 102. ISBN   978-1441965011.