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Scleritis
Matthew J. Schear, DO, MS
Anne Steiner, MD
image 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 52 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
  • Sjögren 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
image DIAGNOSIS
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 (FTAABS), 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
  • P.949

  • 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
image 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 débridement (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.
image 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.
REFERENCES
1. 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.
2. Hodson KL, Galor A, Karp CL, et al. Epidemiology and visual outcomes in patients with infectious scleritis. Cornea. 2013;32(4):466-472.
3. Beardsley RM, Suhler EB, Rosenbaum JT, et al. Pharmacotherapy of scleritis: current paradigms and future directions. Expert Opin Pharmacother. 2013;14(4):411-424.
4. Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, et al. Scleritis therapy. Ophthalmology. 2012;119(1):51-58.
5. 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.
6. 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
&NA;
  • 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
&NA;
ICD10
  • H15.009 Unspecified scleritis, unspecified eye
  • H15.019 Anterior scleritis, unspecified eye
  • H15.039 Posterior scleritis, unspecified eye
Clinical Pearls
&NA;
  • 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.