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Practice Essentials
Background
The correct and rapid diagnosis and the appropriate systemic therapy can
halt the relentless progression of both ocular and systemic processes, thus
preventing destruction of the globe while prolonging survival and improving
quality of life.
Scleritis may be classified into anterior and posterior. Anterior scleritis can
be diffuse, nodular, necrotizing with inflammation (necrotizing), and
necrotizing without inflammation (scleromalacia perforans). The most
common clinical forms are diffuse scleritis and nodular scleritis. Necrotizing
scleritis with or without inflammation is much less frequent, more ominous,
and frequently associated with systemic autoimmune disorders, as well as
peripheral ulcerative keratitis. Posterior scleritis is characterized by
flattening of the posterior aspect of the globe, thickening of the posterior
coats of the eye (choroid and sclera), and retrobulbar edema.
Pathophysiology
Epidemiology
Frequency
United States
The exact incidence of scleritis is uncertain, although scleritis is not
common. The reported prevalence of scleritis is skewed by the somewhat
selected referrals of the reporting institutions.
Mortality/Morbidity
Patients with scleritis are at risk for ocular complications and systemic disease
association.
Disease association may be found in about 57% of patients with scleritis. Up to 48%
of patients with scleritis present with a known connective-tissue or vasculitic disease.
Some of these diseases are potentially lethal unless treated with prompt and
aggressive therapy. Other patients may present with concomitant trauma, infection,
or postsurgical inflammation. Systemic disease association is most common in cases
of necrotizing scleritis. Scleritis may be the first manifestation of a potentially lethal
systemic disease.
Sex
Women are more likely to have scleritis than men (1.6:1).
Age
Scleritis is most common in the fourth to sixth decades of life. The peak
incidence of scleritis is in the fifth decade.
Prognosis
Clinical Presentation
History
Pain is the most common symptom for which patients seek medical assistance, and
it is the best indicator of active inflammation. Pain results from both direct stimulation
and stretching of the nerve endings by the inflammation.
The following pain descriptions are characteristic of scleritis:
Severe, penetrating pain that radiates to the forehead, brow, jaw, or sinuses
Upon palpation, the patient may describe tenderness that is diffuse with possible
radiation to other parts of the head.
Redness gradually increases over several days. It has a bluish red tinge, which is
seen best when the examination is performed in natural light, not through the slit
lamp. It may be localized in one sector or involve the whole sclera; most frequently, it
is in the interpalpebral area. This discoloration does not blanche after topical
applications of routine sympathomimetic dilating agents (Neo-Synephrine 2.5%).
Past medical and ocular histories may elucidate systemic diseases, trauma, drugs,
or surgical procedures that might cause scleritis:
Infectious diseases
Past ocular surgical procedures, especially within a year prior to the onset of
scleritis, might be significant.
Respiratory
Cardiac
Genitourinary
Rheumatologic
Gastrointestinal
Neurologic
Pulmonary
Physical
The head and extremities (eg, nose, mouth, external ear, skin, joints)
examinations may reveal significant signs, which might be compatible with
a particular underlying disease. An eye examination might detect and
characterize scleral disease. Include scleral and general eye examinations.
Scleral examination
Daylight
The sclera may appear diffuse, deep bluish red, or violaceous. After several attacks
of scleral inflammation, areas of scleral thinning and translucency may appear,
allowing the dark uvea to show.
Extraocular muscles
Lids and orbit
Cornea
Uvea
Lens
Intraocular pressure (IOP)
Dilated fundus
Causes
Scleritis may occur isolated (43%) or in association with several types of
disorders (57%), as follows:
Autoimmune (48%)
o
Connective-tissue diseases and other inflammatory conditions
include the following [3] :
Rheumatoid arthritis
Ankylosing spondylitis
Reactive arthritis
Psoriatic arthritis
Gouty arthritis
Relapsing polychondritis
Polymyositis
Sjgren syndrome
Polyarteritis nodosa
Behet disease
Cogan syndrome
Laboratory Studies
Imaging Studies
Necrotizing scleritis
Medical Care
Necrotizing scleritis
The initial therapy consists of immunosuppressive drugs that are supplemented with
corticosteroids during the first month; the latter is tapered slowly, if possible.
Cyclophosphamide should be the first choice in treating patients with an underlying
systemic vasculitis such as granulomatosis with polyangiitis or polyarteritis nodosa.
In case of therapeutic failure, biologic response modifiers, such as infliximab or
adalimumab, may be effective. Other alternatives are golimumab, certolizumab,
tocilizumab, and rituximab, although their efficacy awaits further study. [20, 21]
Periocular steroid injections should not be applied in cases of necrotizing scleritis or
peripheral ulcerative keratitis but could be very helpful in diffuse or nodular scleritis
as an adjunctive therapy. Some authors believe that depot steroids actually may
exacerbate necrotizing disease.
Surgical Care
Tectonic surgical procedures rarely may be required to preserve the integrity of the
globe.
Scleral grafts from fresh donor sclera or glycerin-preserved sclera are available
through eye banks. Grafts may be performed in cases of pending perforation during
the time before the effects of systemic immunosuppressive agents manifest.
Corneal tissue may be used for associated corneal disease.
Consultations
See the list below:
Precautions
Medication Summary
For relief of mild to moderate pain; inhibits inflammatory reactions and pain
by decreasing activity of cyclooxygenase, which results in a decrease of
prostaglandin synthesis.
Piroxicam (Feldene)
Prognosis