Collagen vascular diseases
Herpes zoster, herpes simplex, syphilis
Acute onset of redness, mild irritation, tearing, rarely, pain
Sectorial or diffuse hyperemia, primarily involving the middle episcleral plexus, with some secondary involvement of the overlying conjunctival vessels (Fig. 9-1)
The inflamed episcleral vessels have a straight, radial configuration.
Topical phenylephrine 2.5% will cause blanching and enhance visualization of the normal deep vascular plexus over the sclera.
Somewhat tender, usually solitary, localized injected nodule that can be moved slightly over the sclera
A localized area of corneal thinning secondary to desiccation (delle) may develop adjacent to an episcleral nodule.
Conjunctivitis: usually associated with papillary or follicular response in the tarsal conjunctiva. May have a mucoid or purulent discharge
Scleritis: Pain is deeper and more severe, sclera has a purple or bluish hue under natural light, injected scleral vessels have criss-crossing configuration, vessels are immobile over the globe, patients tend to be older.
Inflamed pinguecula: located in the completely mobile conjunctiva
Examination is made with the slit lamp and under natural or room light.
Attempt to move the episcleral vessels over the sclera using a cotton-tipped stick under topical anesthesia.
Apply phenylephrine 2.5% and observe for blanching of episcleral vessels after 15 minutes.
Systemic work-up if history is suggestive of collagen vascular disease or gout.
Artificial tears and cool compresses q.i.d.
Consider a short course of a topical NSAID (e.g., diclofenac q.i.d., ketorolac q.i.d., bromfenac q.d. or b.i.d., or nepafenac t.i.d.).
Oral NSAID (e.g., ibuprofen 400 to 600 mg t.i.d. to q.i.d., indomethacin 12.5 to 25 mg q.d. to t.i.d. or flurbiprofen 100 mg b.i.d. to t.i.d.) for moderate to severe cases.
Consider a short course of topical corticosteroids (e.g., fluorometholone 0.1% to 0.25%, loteprednol 0.2% to 0.5%) q.i.d. in recalcitrant cases. Rarely, oral corticosteroids are required.
Good to very good. Episcleritis is often a recurrent condition.
Scleritis is a severe, potentially sight-threatening ocular disorder that has a totally different prognosis than episcleritis. It may be mild and benign or severe and destructive. Females are affected more often than males, and the condition is frequently bilateral. Most scleritis affects the anterior sclera. Anterior scleritis can be divided into the clinical forms described below.
Diffuse: diffuse hyperemia and distortion of the pattern of the deep vascular plexus, associated with variable episcleral and conjunctival injection (Fig. 9-2A). This is the most benign form and is associated with the least severe systemic conditions.
Nodular: Tender, usually solitary, deep, localized injected nodule that cannot be moved over the sclera (Fig. 9-2B)
This is the most destructive form of scleritis. It is associated with ocular or systemic complications in 60% of patients, and 40% may have loss of vision. One-third of patients may die within a few years as a result of severe autoimmune disease if they are inadequately treated.
Gradual appearance of painful, localized, avascular patch overlying an area of scleral necrosis (Fig. 9-2C)
Inflammation may be localized to the surrounding sclera or may become diffuse.
The underlying uvea becomes progressively visible through the thinned and necrotic sclera.
Without inflammation (scleromalacia perforans)
Typically seen in patients with long-standing rheumatoid arthritis
Asymptomatic development of enlarging gray-blue patches of scleral thinning
Exposure of underlying uvea through areas of thin, devitalized sclera with large bridging vessels
Anterior scleral staphylomas can occur.
Half of patients with scleritis have an associated systemic disease.
Surgery (e.g., cataract surgery, trabeculectomy, scleral buckle) (Fig. 9-2D)
Topical medications (e.g., NSAIDs, corticosteroids) (Fig. 9-2E)
Collagen vascular diseases
Rheumatoid arthritis (most common)
Wegener’s granulomatosis (moderately common)
Others (e.g., systemic lupus erythematosus, juvenile rheumatoid arthritis, juvenile chronic arthritis, polymyositis)
Herpes zoster ophthalmicus (moderately common)
Stromal keratitis, peripheral corneal melt and sclerosing keratitis, associated with worse prognosis
Posterior uveitis, posterior scleritis, exudative retinal detachment
Severe, boring pain that may radiate to the orbit or head and awaken patients from sleep; redness; tearing; photophobia; decreased vision
Onset is generally gradual but may be acute.
History of associated systemic diseases in many cases
Inflamed conjunctiva, episclera, and sclera. Scleral vessels have criss-crossing patterns, do not blanch with 2.5% phenylephrine drops, and are immobile over the globe.
Sclera has a purple-bluish hue when examined grossly under natural light.
Scleral inflammation can be sectorial or diffuse. Scleral edema, nodule, or thinning and necrosis may be seen. Sometimes, the sclera may become more translucent without significant thinning.
Anterior chamber reaction
Other signs of ocular complications as mentioned above may be observed.
Episodes of resolved scleritis can result in blue-gray areas of thinned sclera (Fig. 9-2F).
Examine with the slit lamp and under natural or room light.
Attempt to move the injected vessels over the sclera using a cotton-tipped stick under topical anesthesia.
Apply phenylephrine 2.5% and observe for absence of blanching of the scleral vessels after 15 minutes.
Consider cultures if there is an overlying conjunctival epithelial defect.
Investigate for associated systemic diseases.
Nonnecrotizing diffuse and nodular scleritis
Oral NSAIDs (e.g., indomethacin
25 mg t.i.d. or flurbiprofen 100 mg t.i.d.) are often effective.
For more resistant cases, systemic corticosteroids (e.g., prednisolone 1 to 2 mg/kg/d PO q.d.), then tapered over the next few months
Consider oral NSAIDs for 1 week.
If there is no improvement, systemic corticosteroid can be added as above.
For severe disease, oral corticosteroids can be initiated as above, or the patient can be hospitalized and treated with IV corticosteroids.
In resistant cases, other immunosuppressive therapy may be indicated (e.g., methotrexate, azathioprine, mycophenolate mofetil, cyclosporine, and cyclophosphamide). Biologics may be helpful in recalcitrant disease (e.g., infliximab, adalimumab, or rituximab).
Scleral (or pericardial or dural) patch graft may be required if there is a risk of perforation.
Topical corticosteroids are not effective
for scleritis. Topical cyclosporine 2% drops,
4 to 6 drops per day, may be of limited benefit.
Subtenon’s corticosteroid injections are occasionally used, but they may cause scleral thinning and may increase the risk of perforation.
Good for nonnecrotizing scleritis, guarded for necrotizing scleritis. The prognosis depends greatly on whether the underlying systemic disease can be identified and treated adequately.