Scleritis

Aka: Scleritis, Nodular Scleritis, Necrotizing Scleritis

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II. Epidemiology

  1. Rare

III. Pathophysiology

  1. Severe Scleral inflammation
  2. Variations
    1. Nodular Scleritis
    2. Necrotizing Scleritis (most destructive)
    3. Anterior Scleritis (deep to Conjunctiva)
    4. Posterior Scleritis (overlying Retina)

IV. Causes

  1. Idiopathic in 50% of cases
  2. Connective Tissue Disease
    1. Rheumatoid Arthritis (most common cause)
      1. Accounts for up to one third of Scleritis cases
      2. Scleritis occurs in 4-10% of RA cases
    2. Granulomatosis with Polyangiitis (previously known as Wegener's Granulomatosis)
    3. Polyarteritis Nodosa
    4. Systemic Lupus Erythematosus
    5. Relapsing Polychondritis
    6. Reiter's Syndrome
    7. Psoriatic Arthritis
    8. Ankylosing Spondylitis
  3. Inflammatory Bowel Disease
    1. Crohn's Disease
    2. Ulcerative Colitis
  4. Infectious (uncommon)
    1. Herpes Zoster
    2. Herpes Simplex Virus
    3. Pseudomonas
    4. Aspergillus
    5. Tuberculosis

V. Symptoms

  1. Red Eye involving one or both eyes
  2. Blurred Vision
  3. Photophobia
  4. Subacute course with gradual onset
  5. Significant Eye Pain (especially Necrotizing Scleritis)
    1. Deep boring toothace-type Eye Pain
    2. Pain radiates to eyebrows, cheeks and temples
    3. Pain worse with eye movments
    4. Intense night pain with pain on awakening
  6. Assocated symptoms
    1. Fever
    2. Headache
    3. Vomiting

VI. Signs

  1. Decreased Visual Acuity
  2. Pain on palpation
  3. Diffuse Eye Redness
  4. Scleral edema
  5. Corneal Ulceration
  6. Scleromalacia (severe cases)
    1. Sclera thins and takes on a bluish hue

VII. Signs: Slit Lamp Exam

  1. Critical to do this exam prior to Fluorescein application
    1. Fluorescein can settle in the stroma and obscure the Scleritis findings and extent
  2. Localized, raised hyperemia of Sclera
  3. Elevated Scleral vessels
  4. Scleritis does not blanch with topical Phenylephrine
    1. Phenylephrine blurs Vision for 3 hours
    2. Phenylephrine contraindicated in Glaucoma
  5. Avascular areas over Sclera

VIII. Associated Conditions

  1. Associated with Rheumatologic Conditions, Inflammatory Bowel Disease in >50% of cases
  2. Anterior Scleritis
    1. Iritis
    2. Glaucoma
  3. Posterior Scleritis
    1. Retinal Detachment
    2. Proptosis

IX. Differential Diagnosis

  1. See Red Eye
  2. Episcleritis
  3. Conjunctivitis

X. Management

  1. NSAIDs
    1. Indomethacin 25 mg orally twice daily
    2. Ibuprofen 600 mg orally three times daily
    3. Naproxen 250 to 500 mg orally twice daily
  2. Ophthalmology referral
  3. Advanced cases may require Immunosuppressants and Corticosteroids

XI. Course

  1. Duration of months to years

XII. Complications

  1. Scleral thinning or perforation
  2. Staphyloma
  3. Scleromalacia perforans (in Rheumatoid Arthritis)

XIII. References

  1. Goldstein in Yanoff (1999) Ophthalmology, p. 13.1
  2. Ruddy (2001) Kelley's Rheumatology, Saunders, p. 396
  3. Nakla (1998) Gastroenterol Clin North Am 27:697-711 [PubMed]
  4. Pflipsen (2016) Am Fam Physician 93(12): 991-8 [PubMed]

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