Introduction
Background
Scleritis is an inflammatory disease that affects the sclera; it may be localized, nodular, or diffuse. It may involve the anterior (visible segment) and/or posterior segments of the eye and manifests with redness of the eye and severe eye pain. Isolated posterior scleritis will not present with redness of the visible portion of the eye and may or may not present with pain.
The 4 types of anterior scleritis are as follows:
- Diffuse anterior scleritis: This is characterized by widespread inflammation of the anterior portion of the sclera. This is the most common form of anterior scleritis as well as the most benign.
- Nodular anterior scleritis: This type is characterized by one or more erythematous, immovable, tender inflamed nodules on the anterior sclera. Approximately 20% of cases progress to necrotizing scleritis.
- Necrotizing anterior scleritis with inflammation: This form frequently accompanies serious systemic collagen vascular disorders including rheumatoid arthritis. Pain with this condition is usually extreme, and damage to the sclera is often marked. Necrotizing anterior scleritis with corneal inflammation is also known as sclerokeratitis.
- Necrotizing anterior scleritis without inflammation: This type most frequently occurs in patients with long-standing rheumatoid arthritis; it is due to the formation of a rheumatoid nodule in the sclera and is notable for its absence of symptoms. Necrotizing anterior scleritis without inflammation is also known as scleromalacia perforans.
Posterior scleritis occurs much less frequently than anterior scleritis, but they may occur concurrently.
In cases of non-necrotizing scleritis, vision is often maintained unless complications such as uveitis occur.
The examiner must be cognizant of conditions that masquerade as scleritis such as toxoplasmosis-induced posterior uveitis and chronic lymphocytic leukemia. Episcleritis often presents similarly to scleritis, but inflammation and erythema is isolated to the episclera, which lies between the sclera and the conjunctiva. Episcleritis has a more benign course and does not cause any visual changes or permanent impairment; however, recurrence is common.
Pathophysiology
The sclera, which consists of collagen and elastic connective tissue, provides a tough protective casing around the eye. Enzymatic degradation of collagen fibrils and invasion of inflammatory cells, including T cells and macrophages, appear to play an important role.
The thickness of the sclera varies from 0.3-1.2 mm. Healthy sclera is consistently white. Inflammation, the principal pathology affecting the sclera, is frequently part of a general inflammatory reaction associated with a systemic immune-mediated collagen vascular disease.
Inflammation of the sclera can progress to ischemia and necrosis, eventually leading to scleral thinning and perforation of the globe. Necrotizing anterior scleritis represents a particularly destructive form of scleritis.
Frequency
United States
Scleritis is an uncommon disease. Well-defined incidence rates are hard to find. The prevalence is estimated to be 6 cases per 10,000 population. Of patients diagnosed with scleritis, anterior scleritis is demonstrated in 94% of patients, as opposed to posterior scleritis, which is diagnosed only 6% of the time.
An increased incidence of scleritis has been reported in patients taking bisphosphonates, which are commonly used in the management of osteoporosis.
International
No particular geographic distribution has been noted.
Mortality/Morbidity
- Morbidity arises from primary scleritis and associated systemic disease.
- Giant pigment epithelial tear and retinal detachment has been reported in a patient with scleritis.
- A significant percentage of patients with concurrent scleritis and collagen vascular disease die within 5 years.
- In 15% of cases, scleritis is the presenting manifestation of collagen vascular disorder and may precede additional symptoms by one to several months.
Race
No published information is available on racial differences.
Sex
The female-to-male ratio is approximately 1.6:1.
Age
- Cases have been reported in patients ranging from 11-87 years of age. Mean age for all types of scleritis is 52 years.
- Episcleritis tends to affect younger patients.
Clinical
History
- Pain
- Severe, constant, deep, boring, or pulsating pain is noted.
- Pain worsens with movement of the eye.
- Pain is worse at night and may awaken the patient.
- Pain may be referred to the eyebrow, temple, or jaw. Occasionally, the configuration of the pain pattern corresponds to the course of the trigeminal nerve.
- Onset: Scleritis is subacute, a more gradual onset occurs than is seen in episcleritis.
- Duration of symptoms: Scleritis generally persists from months to years, whereas episcleritis usually resolves within weeks.
- Associated symptoms
- Erythema of the eye is a defining symptom with anterior scleritis.
- Lacrimation
- Photophobia
- Discharge is ordinarily not part of the clinical picture of scleritis.
- Less prevalent symptoms
- Fever
- Nausea/vomiting
- Headache
- Unilateral or bilateral: Both eyes are affected in slightly more than one half of cases.
- Similar previous episodes
- Prior history
- Underlying systemic disease
- Trauma
- Ocular surgery
- Glaucoma
- Exposure to irritants/chemicals
- Previous use of eye drops
- Medications
Physical
- As with any eye complaint (except chemical injury), begin with vision testing.
- Visual acuity may be normal or decreased with all forms of scleritis.
- Visual impairment is most pronounced with posterior scleritis.
- Additionally, a complete physical examination, particularly of the skin, joints, heart, and lungs, may be obligatory when an underlying complicating illness is suspected.
- External examination
- Ensure that the eye examined is supported by satisfactory lighting, inspecting for breadth and degree of injection, as well as the presence of bluish hue signifying attenuation of the sclera.
- Prominent findings may include photophobia, tearing without discharge, tenderness of the eye, and purplish red, edematous, engorged blood vessels.
- Deeper sclera blood vessels appear darker, follow a radial pattern, and do not move when manipulated with a cotton swab.
- Administration of topical phenylephrine 2.5-10% causes blanching of the more superficial episcleral vessels but does not change the engorgement of deeper sclera vessels and can help differentiate between scleritis and episcleritis.
- Perform a slit lamp/biomicroscopic examination to judge the depth and breadth of involvement.
- Determine whether there is diffuse or segmental involvement. Widespread injection of the conjunctival and deep scleral vessels is characteristic of diffuse anterior scleritis.
- Localized elevation of the sclera is representative of nodular anterior scleritis.
- Nodules in anterior scleritis are immobile, differing from the non-mobile nodules that can be seen in episcleritis.
- Scleral thinning is suggested when the choroid pigment becomes a blue-violet hue best seen in natural lighting.
- Global perforation typically results in an abnormal shape of the pupil and/or uveal or vitreous prolapse.
- Use of a red-free filter (green light) helps identify avascular areas of the sclera. Corneal changes are present in up to 50% of cases.
- Examination of the eyelids for possible blepharitis or conjunctivitis should be performed.
- Assessment of the anterior chamber for possible narrowing, hyphema, or hypopyon. Angle narrowing can be seen in acute angle-closure glaucoma. Hyphema can be seen with trauma and/or bleeding disorders. Hypopyon may be seen in uveitis/iritis.
- Posterior scleritis
- External findings associated with posterior scleritis include restriction of eye movements, sensitivity to palpation, and proptosis.
- Dilation of the fundus may be necessary to identify posterior scleritis. Posterior scleritis may simulate amelanotic choroidal.
- Funduscopic examination of the patient with posterior scleritis may also reveal papilledema, choroidal folds, and retinal hemorrhage or detachment.
Causes
Scleritis coexists with a serious systemic disease in almost one half of cases; the underlying problem is frequently a connective tissue disorder.
- Rheumatoid arthritis is the underlying disease for approximately one sixth of patients suffering from scleritis, and approximately 1% of patients with rheumatoid arthritis will develop scleritis at some point in the course of the disease. Scleritis associated with RA is due to the development of a rheumatoid nodule on the sclera and is associated with an increased risk of mortality.
- Other connective tissue and autoimmune diseases seen with scleritis include the following:
- Systemic lupus erythematosus (SLE)
- Polyarteritis nodosa
- Seronegative spondyloarthropathies
- Ankylosing spondylitis
- Psoriatic arthritis
- Reactive arthritis
- Wegener granulomatosis
- Relapsing polychondritis
- Sarcoidosis
- Inflammatory bowel disease
- Sjörgen syndrome
- Additional maladies that may accompany scleritis include the following:
- Syphilis
- Post herpes zoster ophthalmicus
- Tuberculosis
- Gout
- Lyme disease
- Foreign body
- Hypertension
More on Scleritis |
 Overview: Scleritis |
| Differential Diagnoses & Workup: Scleritis |
| Treatment & Medication: Scleritis |
| Follow-up: Scleritis |
| References |
| Next Page » |
References
Ahmadi-Simab K, Lamprecht P, Nölle B, Ai M, Gross WL. Successful treatment of refractory anterior scleritis in primary Sjogren's syndrome with rituximab. Ann Rheum Dis. Jul 2005;64(7):1087-8. [Medline].
Ashok D, Ayliffe WH, Kiely PD. Necrotizing scleritis associated with rheumatoid arthritis: long-term remission with high-dose infliximab therapy. Rheumatology (Oxford). Jul 2005;44(7):950-1. [Medline].
Bauer AM, Fiehn C, Becker MD. Celecoxib, a selective inhibitor of cyclooxygenase 2 for therapy of diffuse anterior scleritis. Am J Ophthalmol. Jun 2005;139(6):1086-9. [Medline].
Bonfioli AA, Orefice F. Toxoplasmosis. Semin Ophthalmol. Jul-Sep 2005;20(3):129-41. [Medline].
Boonman ZF, de Keizer RJ, Watson PG. Smoking delays the response to treatment in episcleritis and scleritis. Eye. Sep 2005;19(9):949-55. [Medline].
Burton BJ, Cunningham ET Jr, Cree IA, Pavesio CE. Eye involvement mimicking scleritis in a patient with chronic lymphocytic leukaemia. Br J Ophthalmol. Jun 2005;89(6):775-6. [Medline].
Cazabon S, Over K, Butcher J. The successful use of infliximab in resistant relapsing polychondritis and associated scleritis. Eye. Feb 2005;19(2):222-4. [Medline].
Deokule S, Saeed T, Murray PI. Deep intramuscular methylprednisolone treatment of recurrent scleritis. Ocul Immunol Inflamm. Feb 2005;13(1):67-71. [Medline].
Fahim K, Houghton O, Dastjerdi M, Mian SI. Posterior scleritis secondary to Pseudomonas aeruginosa keratitis. Cornea. Oct 2005;24(7):879-81. [Medline].
Fong LP, Sainz de la Maza M, Rice BA, Kupferman AE, Foster CS. Immunopathology of scleritis. Ophthalmology. Apr 1991;98(4):472-9. [Medline].
Legmann A, Foster CS. Noninfectious necrotizing scleritis. Int Ophthalmol Clin. Winter 1996;36(1):73-80. [Medline].
Leung S, Ashar BH, Miller RG. Bisphosphonate-associated scleritis: a case report and review. South Med J. Jul 2005;98(7):733-5. [Medline].
McCluskey P, Wakefield D. Intravenous pulse methylprednisolone in scleritis. Arch Ophthalmol. Jun 1987;105(6):793-7. [Medline].
McMullen M, Kovarik G, Hodge WG. Use of topical steroid therapy in the management of nonnecrotizing anterior scleritis. Can J Ophthalmol. Jun 1999;34(4):217-21. [Medline].
Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Surv Ophthalmol. Jul-Aug 2005;50(4):351-63. [Medline].
Sainz de la Maza M, Jabbur NS, Foster CS. An analysis of therapeutic decision for scleritis. Ophthalmology. Sep 1993;100(9):1372-6. [Medline].
Sainz de la Maza M, Jabbur NS, Foster CS. Severity of scleritis and episcleritis. Ophthalmology. Feb 1994;101(2):389-96. [Medline].
Smith JR, Mackensen F, Rosenbaum JT. Therapy insight: scleritis and its relationship to systemic autoimmune disease. Nat Clin Pract Rheumatol. Apr 2007;3(4):219-26. [Medline]. [Full Text].
Thill M, Richard G. Giant pigment epithelial tear and retinal detachment in a patient with scleritis. Retina. Jul-Aug 2005;25(5):667-8. [Medline].
Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. Mar 1976;60(3):163-91. [Medline].
Wirbelauer C. Management of the red eye for the primary care physician. Am J Med. Apr 2006;119(4):302-6. [Medline]. [Full Text].
Further Reading
Keywords
scleritis, sclera, leucitis, anterior scleritis, posterior scleritis, necrotizing anterior scleritis, scleromalacia perforans, diffuse anterior scleritis, eye redness, eye pain, sclerokeratitis
