II. Epidemiology
- Rare
III. Pathophysiology
- Severe Scleral inflammation
- Variations
- Nodular Scleritis
- Necrotizing Scleritis (most destructive)
- Anterior Scleritis (deep to Conjunctiva)
- Posterior Scleritis (overlying Retina)
IV. Causes
- Idiopathic in 50% of cases
-
Connective Tissue Disease
-
Rheumatoid Arthritis (most common cause)
- Accounts for up to one third of Scleritis cases
- Scleritis occurs in 4-10% of RA cases
- Granulomatosis with Polyangiitis (previously known as Wegener's Granulomatosis)
- Polyarteritis Nodosa
- Systemic Lupus Erythematosus
- Relapsing Polychondritis
- Reiter's Syndrome
- Psoriatic Arthritis
- Ankylosing Spondylitis
-
Rheumatoid Arthritis (most common cause)
- Inflammatory Bowel Disease
- Infectious (uncommon)
V. Symptoms
- Red Eye involving one or both eyes
- Blurred Vision
- Photophobia
- Subacute course with gradual onset
- Significant Eye Pain (especially Necrotizing Scleritis)
- Deep boring toothace-type Eye Pain
- Pain radiates to eyebrows, cheeks and temples
- Pain worse with eye movments
- Intense night pain with pain on awakening
- Assocated symptoms
VI. Signs
- Decreased Visual Acuity
- Pain on palpation
- Diffuse Eye Redness
- Scleral edema
- Corneal Ulceration
- Scleromalacia (severe cases)
- Sclera thins and takes on a bluish hue
VII. Signs: Slit Lamp Exam
- Critical to do this exam prior to Fluorescein application
- Fluorescein can settle in the stroma and obscure the Scleritis findings and extent
- Localized, raised hyperemia of Sclera
- Elevated Scleral vessels
- Scleritis does not blanch with topical Phenylephrine
- Phenylephrine blurs Vision for 3 hours
- Phenylephrine contraindicated in Glaucoma
- Avascular areas over Sclera
VIII. Associated Conditions
- Associated with Rheumatologic Conditions, Inflammatory Bowel Disease in >50% of cases
- Anterior Scleritis
- Posterior Scleritis
IX. Differential Diagnosis
X. Management
-
NSAIDs
- Indomethacin 25 mg orally twice daily
- Ibuprofen 600 mg orally three times daily
- Naproxen 250 to 500 mg orally twice daily
- Ophthalmology referral
- Advanced cases may require Immunosuppressants and Corticosteroids
XI. Course
- Duration of months to years
XII. Complications
- Scleral thinning or perforation
- Staphyloma
- Scleromalacia perforans (in Rheumatoid Arthritis)
XIII. References
- Goldstein in Yanoff (1999) Ophthalmology, p. 13.1
- Ruddy (2001) Kelley's Rheumatology, Saunders, p. 396
- Nakla (1998) Gastroenterol Clin North Am 27:697-711 [PubMed]
- Pflipsen (2016) Am Fam Physician 93(12): 991-8 [PubMed]
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Related Studies
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 |