AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

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:

1. 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.
2. 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.
3. 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.
4. 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.

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

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

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

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Other Problems to be Considered

Cerebral tumor
Chronic lymphocytic leukemia
Immunosuppression
Collagen vascular disease

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Lab Studies

• Depending on the clinical suspicion, laboratory studies may be warranted. Laboratory tests include, but are not limited to, the following:
• • Complete blood count (CBC) and electrolytes
  • Erythrocyte sedimentation rate (ESR)
  • FTA-ABS (RPR)
  • Uric acid
  • Rheumatoid factor
  • Antinuclear antibody (ANA)
• The ED is the ideal location to initiate evaluation for collagen vascular disease, infection (conjunctival cultures), and immunocompetency. If scleritis is suspected, the emergency physician will contact an ophthalmologist. This would be a good opportunity to establish an evaluation and intervention plan. Getting the laboratory studies in the ED will save time for the patient and practitioners.

Imaging Studies

• B-scan ultrasonography may assist in detecting posterior scleritis. MRI or CT scans may play a role, but they should be ordered in consultation with an ophthalmologist.
• Chest radiography may be indicated to look for underlying pulmonary involvement arising from systemic disease.
• Imaging of sacroiliac joints is prudent when ankylosing spondylitis is suspected.

Other Tests

• Instillation of phenylephrine, a mydriatic vasoconstrictor, helps differentiate deep scleral episcleral blood vessel involvement from superficial involvement; superficial vessels blanch following application of phenylephrine, while deeper vessels remain unaffected.
• Seidel test helps detect possible global perforation. Apply a moistened fluorescein strip over the potential site of perforation while viewing under a slit lamp. If perforation exists, the fluorescein dye becomes diluted by the aqueous humor, appearing as a green dilute stream within the dark, concentrated, orange dye.

Procedures

• Apply an eye shield when scleral thinning or global perforation is suspected.  
• • Ensure that the eye guard does not contact the eyelid or globe.
  • Apply tape from the forehead to zygoma.
  • In the absence of an eye shield, use a paper or polystyrene cup, provided it is large enough to cover the eye without placing undue pressure on the globe.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Prehospital Care

• Primary complaint of atraumatic eye pain rarely necessitates prehospital care, other than expedient transport to the ED.
• Take care not to overlook serious comorbidity.
• If global perforation is suspected, shield the eye and avoid palpation.

Emergency Department Care

• Recognition of the problem and timely ophthalmology referral are cornerstones of ED management.
• Pain medications may be prescribed in order to provide comfort for the patient.
• Detection of scleral thinning mandates shielding of the involved eye to decrease risk of perforation.
• Scleritis treatment will require immunomodulator therapy once the definitive diagnosis is made.
• The emergency practitioner can discuss using a topical steroid agent with the ophthalmologist. The caveat being that topical steroid therapy typically fails. However, it could be considered as first-line treatment for nonnecrotizing anterior scleritis, especially in cases in which the likelihood of complications from systemic steroid or nonsteroidal anti-inflammatory drug (NSAID) therapy is high.

Consultations

• Severity of scleritis and depth of involvement determine the urgency of ophthalmology consultation.
• Serious systemic illness may require consultation with a qualified internist and/or rheumatologist.

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Therapeutic goals for scleritis are familiar to any emergency practitioner: relieve the patient’s pain and initiate therapy that will positively alter the course of the disease.

From the patient's perspective, pain cessation may be the most important action taken by the emergency practitioner. Outcome will depend on the patient's response to immunosuppressive therapy.

Narcotics and systemic NSAIDs may render temporary pain relief and can be started in the ED.

NSAIDs are generally found to be effective in approximately one third of patients with diffuse anterior scleritis and two thirds of patients with nodular anterior scleritis. NSAIDs have also been found to be helpful in patients with idiopathic posterior scleritis.
   
Initiation of immunosuppressive therapy may require coordination with an internist and/or rheumatologist.

Topical steroids have a high failure rate but should be discussed with the practitioner who will provide follow-up care.

Subconjunctival steroid injections for non-necrotizing scleritis remain controversial. Localized steroid injections may lead to increased intraocular pressure, scleral melting, or globe perforation/scleral rupture.

Surgical management is generally not required except in rare cases of necrotizing scleritis.

Drug Category: Nonsteroidal anti-inflammatory drugs

These agents are used to decrease pain and inflammation. NSAIDs are thought to act by inhibiting prostaglandin synthesis, interfering with migration of leukocytes, and inhibiting phosphodiesterase.

Drug Category: Immunosuppressive agents

These agents are used in severe (necrotizing scleritis) and resistant forms of the disease. Only an ophthalmologist experienced with the medication should prescribe these drugs.

Drug Category: Glucocorticoids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli and are useful in the treatment of recurrent scleritis.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Further Inpatient Care

• Inpatient care rarely is indicated for scleritis, unless complicated by serious exacerbation of underlying disorder.

Further Outpatient Care

• Referral to an ophthalmologist is mandatory.

In/Out Patient Meds

• A variety of NSAIDs is available to choose from, including diflunisal, naproxen, indomethacin, piroxicam, sulindac, and ibuprofen. These are particularly effective in nodular and diffuse scleritis and ordinarily are prescribed for at least 1 week.
• High-dose oral prednisone is used primarily in necrotizing scleritis and severe nonnecrotizing scleritis.
• Immunosuppressives
• • These agents are used as an adjunct when steroids alone fail to control progression of the disease and include cyclosporine, azathioprine, cyclophosphamide, and methotrexate.
  • These drugs have serious side effects and contraindications and should be prescribed only by a physician who is well aware of their actions.

Transfer

• If global perforation is suspected, transfer to a center with a qualified ophthalmologist may be necessary.

Complications

• Scleral thinning leading to global perforation is the most devastating complication.
• Visual impairment is a possible complication.
• Cornea is affected more than 50% of time. Damage to the cornea may include the following: uveitis, keratitis, glaucoma, and cataracts.
• Posterior chamber derangements may include the following: optic neuritis, choroidal detachment, macular edema, retinal hemorrhage and/or detachment, and papilledema.

Prognosis

• Necrotizing scleritis, the most destructive type of scleritis, and scleritis with extensive scleral thinning or perforation convey less favorable prognoses than other types of scleritis.
• Prognosis of scleritis, when originating from systemic disorders, usually conforms to the course of the underlying disease.

Patient Education

• For excellent patient education resources, visit eMedicine's Eye and Vision Center. Also, see eMedicine's patient education article Eye Pain.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

Medical/Legal Pitfalls

• Failure to differentiate scleritis from the more benign, self-limited episcleritis. A quick bedside test to help distinguish the two entities involves instillation of 1-2 drops of 2.5% phenylephrine into the involved eye; episcleral vessels blanch upon application, while deeper scleral vessels remain unaffected.
• Failure to see discoloration representing scleral thinning because discoloration is overlooked easily in a darkened room
• Failure to document visual acuity before proceeding to any manipulation of the eye or instillation of medications
• Failure to shield the eye if global perforation or scleral thinning is present

ACKNOWLEDGMENTS

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors, Jerome FX Naradzay, MD, Loice Swisher, MD, and Jonathan Adler, MD, to the development and writing of this article.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• References

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Article Last Updated: Apr 14, 2008