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. 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 in >50% of cases
  2. Anterior Scleritis
    1. Iritis
    2. Glaucoma
  3. Posterior Scleritis
    1. Retinal Detachment
    2. Proptosis

IX. Differential Diagnosis

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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Ontology: Necrotizing Scleritis (C0027547)

Concepts Disease or Syndrome (T047)
MSH D015423
SnomedCT 95797003
English Necrotizing Scleritides, Scleritides, Necrotizing, Scleritis, Necrotizing, scleritis necrotizing, scleritis necrotizing (diagnosis), Necrotising scleritis, Necrotizing scleritis, Necrotizing scleritis (disorder), Necrotizing Scleritis
German nekrotisierende Skleritis, Nekrotisierende Skleritis
Japanese 壊死性強膜炎, エシセイキョウマクエン
Portuguese Esclerite Necrosante, Esclerite necrosante
Spanish Escleritis Necrotizante, escleritis necrosante (trastorno), escleritis necrosante, escleritis necrotizante, Escleritis necrosante
French Sclérite nécrosante
Czech Nekrotizující skleritida, nekrotizující skleritida
Hungarian Necrotizáló episcleritis
Norwegian Nekrotiserende skleritt
Italian Sclerite necrotizzante
Dutch necrotiserende scleritis, Necrotiserende scleritis

Ontology: Scleritis (C0036416)

Definition (MSH) Refers to any inflammation of the sclera including episcleritis, a benign condition affecting only the episclera, which is generally short-lived and easily treated. Classic scleritis, on the other hand, affects deeper tissue and is characterized by higher rates of visual acuity loss and even mortality, particularly in necrotizing form. Its characteristic symptom is severe and general head pain. Scleritis has also been associated with systemic collagen disease. Etiology is unknown but is thought to involve a local immune response. Treatment is difficult and includes administration of anti-inflammatory and immunosuppressive agents such as corticosteroids. Inflammation of the sclera may also be secondary to inflammation of adjacent tissues, such as the conjunctiva.
Concepts Disease or Syndrome (T047)
MSH D015423
ICD9 379.00
ICD10 H15.0 , H15.00
SnomedCT 194140003, 155201000, 194139000, 78370002
English Scleritides, Scleritis, SCLERITIS, Scleritis, unspecified, scleritis (physical finding), scleritis, scleritis (diagnosis), Scleritis NOS, Unspecified scleritis, Scleritis [Disease/Finding], Unspecified scleritis (disorder), Inflammation of sclera, Inflammation of white of eye, Scleritis (disorder), Scleritis, NOS
French SCLERITE, Sclérite SAI, Sclérite, non précisée, Sclérite
Portuguese ESCLERITE, Esclerite NE, Esclerite
Spanish ESCLERITIS, Escleritis NEOM, Escleritis no especificada, escleritis no especificada (trastorno), escleritis no especificada, escleritis (trastorno), escleritis, Escleritis
German SKLERITIS, Skleritis NNB, Skleritis, unspezifisch, Skleritis
Dutch scleritis NAO, scleritis, niet-gespecificeerd, scleritis, Scleritis
Italian Sclerite, non specificata, Sclerite NAS, Sclerite
Japanese 強膜炎, 強膜炎、詳細不明, 強膜炎NOS, キョウマクエン, キョウマクエンNOS, キョウマクエン、ショウサイフメイ
Swedish Sklerit
Czech skleritida, Skleritida, Skleritida NOS, Blíže neurčená skleritida
Finnish Skleriitti
Russian EPISKLERIT, NEKROTIZIRUIUSHCHII SKLERIT, SKLERIT, НЕКРОТИЗИРУЮЩИЙ СКЛЕРИТ, СКЛЕРИТ, ЭПИСКЛЕРИТ
Korean 공막염
Polish Zapalenie twardówki, Zapalenie nadtwardówkowe, Zapalenie twardówki martwicze
Hungarian Scleritis, Scleritis k.m.n., Scleritis, nem meghatározott
Norwegian Skleritt

Ontology: Nodular scleritis (C0339194)

Concepts Disease or Syndrome (T047)
SnomedCT 95195003
English nodular scleritis, nodular scleritis (diagnosis), Nodular scleritis, Nodular scleritis (disorder)
Spanish escleritis nodular (trastorno), escleritis nodular