Episcleritis is a benign, self-limiting inflammatory

disease A disease is a particular abnormal condition that negatively affects the structure or function of all or part of an organism, and that is not immediately due to any external injury. Diseases are often known to be medical conditions that a ...

affecting part of the eye called the

episclera The episclera is the outermost layer of the sclera (the white of the eye).Cassin, B. and Solomon, S. ''Dictionary of Eye Terminology''. Gainesville, Florida: Triad Publishing Company, 1990. It is composed of loose, fibrous, elastic tissue and attac ...

. 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. There are two types of episcleritis, nodular and simple. Nodular episcleritis lesions have raised surface. Simple episcleritis lesions are flat. There are two subtypes. In diffuse simple episcleritis, inflammation is generalized. In sectoral simple episcleritis, the inflammation is restricted to one region. Most cases of episcleritis have no identifiable cause, although about a third of cases are associated with various systemic diseases. Often people with episcleritis experience it recurrently. Treatment focuses on decreasing discomfort, and includes lubricating eye drops. More severe cases may be treated with topical corticosteroids or oral anti-inflammatory medications ( NSAIDs).

Signs and symptoms

Symptoms of episcleritis typically include painless redness of the eye (mild pain is possible but atypical), and watery eyes. The pain of episcleritis is typically mild, less severe than in

scleritis 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 ...

, and may be tender to palpation. There are two types of episcleritis: the diffuse type, where the redness involves the entire episclera, and the nodular type, where the redness appears more nodular, involving only a small, well-circumscribed area ( sectoral). The diffuse type of episcleritis may be less painful than the nodular type. Sometimes, small nodules are present within the episclera, which move slightly over the sclera with gentle pressure. Discharge is absent with episcleritis, and vision is unaffected. Patients with episcleritis experience far less

photophobia Photophobia is a medical symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of ...

than patients with

uveitis Uveitis () is inflammation of the uvea, the pigmented layer of the eye 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 in ...

. Episcleritis does not cause the presence of cells or flare in the anterior chamber of the eye. In 80 percent of cases, episcleritis affects only one eye, whereas scleritis often affects both eyes.

Pathophysiology

Episcleritis is caused by inflammation due to the activation of immune cells, including lymphocytes and

macrophages Macrophages (abbreviated as M φ, MΦ or MP) ( el, large eaters, from Greek ''μακρός'' (') = large, ''φαγεῖν'' (') = to eat) are a type of white blood cell of the immune system that engulfs and digests pathogens, such as cancer ce ...

. Most of the time, the cause of episcleritis is never determined (

idiopathic An idiopathic disease is any disease with an unknown cause or mechanism of apparent spontaneous origin. From Greek ἴδιος ''idios'' "one's own" and πάθος ''pathos'' "suffering", ''idiopathy'' means approximately "a disease of its own kin ...

). An identifiable cause ''is'' discovered in about one third of cases. Several diseases are associated with episcleritis, including systemic vasculitis ( polyarteritis nodosa, granulomatosis with polyangiitis,

Behçet's disease Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the ey ...

), connective tissue diseases ( rheumatoid arthritis, relapsing polychondritis, systemic lupus erythematosus), psoriatic arthritis, ankylosing spondylitis,

Cogan syndrome Cogan syndrome (also Cogan's syndrome) is a rare disorder characterized by recurrent inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus (ringing in the ears) and he ...

, rosacea, gout, atopy, Crohn's disease, and

ulcerative colitis Ulcerative colitis (UC) is a long-term condition that results in inflammation and ulcers of the colon and rectum. The primary symptoms of active disease are abdominal pain and diarrhea mixed with blood (hematochezia). Weight loss, fever, and ...

. 59 percent of patients with relapsing polychondritis have either episcleritis or scleritis. Rarely, episcleritis may be caused by

. Very rarely, episcleritis is associated with infections, including Lyme disease, tuberculosis, syphilis, and herpes zoster. The redness in the eye associated with episcleritis is due to engorgement of the large episcleral blood vessels, which run in a radial direction from the limbus. Typically, there is no uveitis, or thickening of the sclera.

Diagnosis

The diagnosis of episcleritis is based upon the history and physical examination. The history should be explored for the presence of the diseases associated with episcleritis, and the symptoms they cause, such as rash, arthritis, venereal disease, and recent viral infection. Episcleritis may be differentiated from scleritis by using

phenylephrine Phenylephrine is a medication primarily used as a decongestant, to dilate the pupil, to increase blood pressure, and to relieve hemorrhoids. In the United States, it was previously used orally as an over-the-counter decongestant to relieve nas ...

or neosynephrine eye drops, which causes blanching of the blood vessels in episcleritis, but not in scleritis. A blue color to the sclera suggests scleritis, rather than episcleritis. After anesthetizing the eye with medication, the conjunctiva may be moved with a cotton swab to observe the location of the enlarged blood vessels. In very rare cases, if episcleritis does not respond to treatment, then a biopsy may be considered, which help provide information regarding any underlying condition (granulomatosis with polyangitis, vasculitis, etc.). However, a biopsy is not routinely necessary in the diagnosis of episcleritis.

Treatment

Often, treatment is not necessary, because episcleritis is a self-limiting condition.

Artificial tears Artificial tears are lubricating eye drops used to relieve dryness and irritation of the ocular surface. Dry eye syndrome ( keratoconjunctivitis sicca) is a common ocular surface disorder and is characterized by disruption of the tear film and i ...

may be used to help with irritation and discomfort. More severe cases can be treated with either topical corticosteroids or oral non-steroidal anti-inflammatory drugs. Ketorolac, a topical NSAID, may be used, but it is not more effective than artificial tears and it causes more side effects.

Prognosis

Episcleritis is a benign, self-limiting condition, meaning patients recover without any treatment. Most cases of episcleritis resolve within 7–10 days. The nodular type is more aggressive and takes longer to resolve. Although rare, some cases may progress to scleritis. However, in general, episcleritis does not cause complications in the eye. Smoking tobacco delays the response to treatment in patients with episcleritis.

Epidemiology

While episcleritis is a common disease, its exact prevalence and incidence are unknown. It typically affects young or middle aged women. The diffuse form of episcleritis (70%) is more common than the nodular form (30%). One retrospective study found 28 percent of individuals with episcleritis experienced recurrent episodes of the disease.

References

External links

{{Eye pathology Disorders of sclera and cornea Inflammations