BASICS
DESCRIPTION
- Scleritis is a painful, inflammatory process of the sclera, part of the eye 's outer coat.
- Categorized into anterior or posterior and diffuse, nodular, or necrotizing
- Commonly associated with systemic disorders
- Frequently requires systemic anti-inflammatory therapy
- Potentially vision-threatening
- In contrast, episcleritis is a self-limited inflammation of the eye with only mild discomfort.
- System(s) affected: ocular
EPIDEMIOLOGY
- Predominant age: most frequently occurs in 4th and 6th decades; mean age for all types of scleritis is ¢ ¼52 years.
- Predominant sex: female > male (1.6:1)
Incidence
- Anterior scleritis, about 94% of cases (1)[B]
- Diffuse anterior scleritis (most common)
- Remaining 6% have posterior scleritis
Prevalence
Estimated to be 6 cases/100,000 people in the general population
ETIOLOGY AND PATHOPHYSIOLOGY
- Frequently associated with a systemic illness (1)[B]
- Most commonly associated with rheumatoid arthritis
- In about 38% of cases, scleritis is the presenting manifestation of an underlying systemic disorder.
- Necrotizing scleritis has the highest association with systemic disease.
- Other etiologies
- Proposed pathogenesis is dependent on type of scleritis. In necrotizing scleritis, the predominant mechanism is likely due to the activity of matrix metalloproteinases.
- Drug-induced scleritis has been reported in patients on bisphosphonate therapy.
- Surgically induced necrotizing scleritis is exceedingly rare and occurs after multiple surgeries.
- Infectious scleritis occurs most commonly after surgical trauma, and Pseudomonas aeruginosa in poorly controlled diabetic patients is the most common causative organism (2)[B].
RISK FACTORS
Individuals with autoimmune disorders are most at risk.
COMMONLY ASSOCIATED CONDITIONS
- Rheumatoid arthritis
- Sj ¶gren syndrome
- Granulomatosis with polyangitis
- Ankylosing spondylitis
- Systemic lupus erythematosus
- Reactive arthritis
- Relapsing polychondritis
- Polyarteritis nodosa
- Sarcoidosis
- Inflammatory bowel disease
- Herpes zoster
- Herpes simplex
- HIV
- Syphilis
- Lyme disease
- Tuberculosis
DIAGNOSIS
HISTORY
- Redness and inflammation of the sclera
- Can be bilateral in about 40% of cases (1)[B]
- Decrease in visual acuity of two or more Snellen lines occurred in about 16% of patients (1)[B].
- Photophobia and tearing
- Pain ranging from mild discomfort to extreme localized tenderness
- May be described as constant, deep, boring, or pulsating
- Pain may be referred to the eyebrow, temple, or jaw.
- Pain may awaken patient from sleep in early hours of morning.
- Severe pain is most commonly associated with necrotizing scleritis (1)[B].
PHYSICAL EXAM
- Examine sclera in all directions of gaze by gross inspection.
- A bluish hue may suggest thinning of sclera.
- Inspect for breadth and degree of injection.
- Check visual acuity.
- Slit-lamp exam using red-free light
- Episcleritis: conjunctival and superficial vascular plexuses displaced anteriorly
- Scleritis: Deep episcleral plexus is the maximum site of vascular congestion, displaced anteriorly d/t edema of underlying sclera. Characteristic blue or violet color, absent in patients with episcleritis
- Ocular tenderness
- Dilated fundus exam to rule out posterior involvement
- A complete physical exam, particularly of the skin, joints, heart, and lungs, should be done to evaluate for associated conditions.
DIFFERENTIAL DIAGNOSIS
- Conjunctivitis
- Episcleritis
- Iritis (anterior uveitis)
- Blepharitis
- Trauma
- Ocular rosacea
- Herpes zoster
DIAGNOSTIC TESTS & INTERPRETATION
- Consider further tests if warranted by history and physical.
- Routine tests to exclude systemic disease
- CBC, serum chemistry, urinalysis, ESR, and/or C-reactive protein
- Specific tests for underlying systemic illness
- Rheumatoid factor, anticyclic citrullinated peptide antibodies, antineutrophil cytoplasmic antibody, and antinuclear antibody may aid in the diagnosis.
- Other tests:
- Fluorescent treponemal antibody absorption (FTA-ABS), rapid plasma reagin, and Lyme titers
- Further imaging studies, such as a chest x-ray and sacroiliac joint films, may be useful if a specific systemic illness is suspected.
- B-scan US to detect posterior scleritis and thickness of sclera
- If indicated, MRI/CT scan to detect orbital disease
Diagnostic Procedures/Other
Biopsy is not routinely required unless diagnosis remains uncertain after above investigations.
Test Interpretation
Different subtypes of scleritis are associated with varying presentations and distinct findings.
- Diffuse anterior scleritis: widespread inflammation
- Nodular anterior scleritis: immovable, inflamed nodule
- Necrotizing anterior scleritis
- "With inflammation " : Sclera becomes transparent.
- Scleromalacia perforans without inflammation: painless and often associated with rheumatoid arthritis
- Posterior scleritis: associated with retinal and choroidal complications, adjacent swelling of orbital tissues may occur
TREATMENT
GENERAL MEASURES
If scleral thinning, glasses/eye shield should be worn to prevent perforation.
MEDICATION
- First-line therapies for noninfectious scleritis (3)[C]
- Oral NSAID therapy, choice based on availability, example is ibuprofen 600 to 800 mg PO TID " QID provided no contraindications exists. About 37% successful (4)[B]
- Topical steroids as adjunct; prednisolone acetate 1% ophthalmic suspension
- Subconjunctival triamcinolone acetonide injection only for nonnecrotizing, 40 mg/mL, 97% improvement after one injection. Increased risk of ocular HTN, cataract, and globe perforation (5)[B]
- Systemic steroids (initial if necrotizing scleritis and preferentially IV if vision threatening, otherwise use if failure of NSAIDs), prednisone 40 to 60 mg PO QD, taper over 4 to 6 weeks
- Antimetabolites including methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, and cyclosporine may be used as steroid-sparing agents. They are generally recommended if steroids cannot be tapered below 10 mg PO QD (4)[C].
- Second-line therapies (4)[C],(5)[B],(6)[A]
- Immunomodulatory agents, infliximab, and adalimumab can be used if patient has failed or is not a candidate for antimetabolites or calcineurin inhibitors. These agents are preferred over etanercept due to higher treatment success.
- Necrotizing anterior scleritis and posterior scleritis
- May require immunosuppressive therapy in addition to systemic steroids
- Treat aggressively due to possible complications if left untreated. May need patch grafting to maintain globe integrity
- Infectious
- Antibiotic therapy resolves about 18% of cases, whereas the remaining often requires surgical intervention such as debridement (2)[B].
ISSUES FOR REFERRAL
- All patients with scleritis should be managed by an ophthalmologist familiar with this condition.
- Rheumatology referral for coexistent systemic disease is helpful for long-term success.
ADDITIONAL THERAPIES
Immunosuppressants used for autoimmune and collagen vascular disorders may be of help in active scleritis.
SURGERY/OTHER PROCEDURES
- In rare cases, scleral biopsy may be indicated to confirm infection or malignancy.
- Ocular perforation requires scleral grafting.
ONGOING CARE
Follow-Up Tests & Special Considerations
- No restrictions
- Avoid contact lens " wear only if there is corneal involvement, which is rare.
Patient Monitoring
- Patient in the active stage of inflammation should be followed very closely by an ophthalmologist to assess the effectiveness of therapy.
- Medication use mandates close surveillance for adverse effects.
DIET
No special diet
PATIENT EDUCATION
Scleritis at PubMed Health: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMHT0024411/
PROGNOSIS
Scleritis is indolent, chronic, and often progressive.
- Diffuse anterior scleritis (best prognosis)
- Necrotizing anterior scleritis (worst prognosis)
- Recurrent bouts of inflammation may occur.
- Scleromalacia perforans has the highest risk of perforation of the globe.
COMPLICATIONS
- Decrease in vision, anterior uveitis, ocular hypertension, and peripheral keratitis (1)[B]
- Cataract and glaucoma can result from disease or treatment with steroids.
- Ocular perforation can occur in severe stages.
REFERENCES
11 Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, et al. Clinical characteristics of a large cohort of patients with scleritis and episcleritis. Ophthalmology. 2012;119(1):43 " 50.22 Hodson KL, Galor A, Karp CL, et al. Epidemiology and visual outcomes in patients with infectious scleritis. Cornea. 2013;32(4):466 " 472.33 Beardsley RM, Suhler EB, Rosenbaum JT, et al. Pharmacotherapy of scleritis: current paradigms and future directions. Expert Opin Pharmacother. 2013;14(4):411 " 424.44 Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, et al. Scleritis therapy. Ophthalmology. 2012;119(1):51 " 58.55 Sohn EH, Wang R, Read R, et al. Long-term, multicenter evaluation of subconjunctival injection of triamcinolone for non-necrotizing, noninfectious anterior scleritis. Ophthalmology. 2011;118(10):1932 " 1937.66 Levy-Clarke G, Jabs DA, Read RW, et al. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology. 2014;121(3):785 " 796.e3.
ADDITIONAL READING
- Doctor P, Sultan A, Syed S, et al. Infliximab for the treatment of refractory scleritis. Br J Ophthalmol. 2010;94(5):579 " 583.
- Iaccheri B, Androudi S, Bocci EB, et al. Rituximab treatment for persistent scleritis associated with rheumatoid arthritis. Ocul Immunol Inflamm. 2010;18(3):223 " 225.
- Rachitskaya A, Mandelcorn ED, Albini TA. An update on the cause and treatment of scleritis. Curr Opin Ophthalmol. 2010;21(6):463 " 467.
- Wakefield D, Di Girolamo N, Thurau S, et al. Scleritis: challenges in immunopathogenesis and treatment. Discov Med. 2013;16(88):153 " 157.
- Wakefield D, Di Girolamo N, Thurau S, et al. Scleritis: immunopathogenesis and molecular basis for therapy. Prog Retin Eye Res. 2013;35:44 " 62.
CODES
ICD10
- H15.009 Unspecified scleritis, unspecified eye
- H15.019 Anterior scleritis, unspecified eye
- H15.039 Posterior scleritis, unspecified eye
- H15.099 Other scleritis, unspecified eye
- H15.059 Scleromalacia perforans, unspecified eye
- H15.051 Scleromalacia perforans, right eye
- H15.003 Unspecified scleritis, bilateral
- H15.002 Unspecified scleritis, left eye
- H15.031 Posterior scleritis, right eye
- H15.012 Anterior scleritis, left eye
- H15.013 Anterior scleritis, bilateral
- H15.001 Unspecified scleritis, right eye
- H15.052 Scleromalacia perforans, left eye
- H15.093 Other scleritis, bilateral
- H15.033 Posterior scleritis, bilateral
- H15.011 Anterior scleritis, right eye
- H15.091 Other scleritis, right eye
- H15.053 Scleromalacia perforans, bilateral
- H15.092 Other scleritis, left eye
- H15.032 Posterior scleritis, left eye
ICD9
- 379.00 Scleritis, unspecified
- 379.03 Anterior scleritis
- 379.07 Posterior scleritis
- 379.09 Other scleritis and episcleritis
- 379.05 Scleritis with corneal involvement
- 379.04 Scleromalacia perforans
SNOMED
- 78370002 Scleritis (disorder)
- 63454000 Anterior scleritis (disorder)
- 267660007 Posterior scleritis (disorder)
- 95797003 Necrotizing scleritis
- 42574005 Scleritis with corneal involvement (disorder)
- 26664005 Scleromalacia perforans (disorder)
CLINICAL PEARLS
- Episcleritis is a self-limited inflammation of the eye with mild discomfort. Scleritis is a painful, severe, and potentially vision-threatening condition. Both conditions can be associated with underlying inflammatory diseases.
- About 35% of all cases of scleritis are associated with a systemic disease such as rheumatoid arthritis. Necrotizing scleritis has the highest association.