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- 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 superficial episclera with only mild discomfort.
- System(s) affected: ocular
- Mean age is 54 years (range 12 to 96).
- Predominant sex: female > male (1.6:1)
Estimated to be 6 cases per 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].
Individuals with autoimmune disorders are most at risk.
Commonly Associated Conditions
- Rheumatoid arthritis (most common)
- Sjögren syndrome
- Granulomatosis with polyangiitis
- HLA-B27–associated ankylosing spondylitis
- Systemic lupus erythematosus
- Reactive arthritis
- Behçet disease
- Juvenile idiopathic arthritis
- Cogan disease
- Relapsing polychondritis
- Polyarteritis nodosa
- Inflammatory bowel disease
- Herpes zoster
- Herpes simplex
- Lyme disease
- IgG4-related disease