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Scleritis

BASICS

DESCRIPTION

- 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].

- 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].

  • 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 necrotizingCommonly associated with systemic disordersFrequently requires systemic anti-inflammatory therapyPotentially 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 arthritisIn about 38% of cases, scleritis is the presenting manifestation of an underlying systemic disorder.Necrotizing scleritis has the highest association with systemic disease.
  • Other etiologiesProposed 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 scleraCan 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 tendernessMay be described as constant, deep, boring, or pulsatingPain 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 lightEpiscleritis: conjunctival and superficial vascular plexuses displaced anteriorlyScleritis: 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 diseaseCBC, serum chemistry, urinalysis, ESR, and/or C-reactive protein
  • Specific tests for underlying systemic illnessRheumatoid 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 suspensionSubconjunctival 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 weeksAntimetabolites 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 scleritisMay require immunosuppressive therapy in addition to systemic steroidsTreat aggressively due to possible complications if left untreated. May need patch grafting to maintain globe integrity
  • InfectiousAntibiotic 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.