Ophthalmology · Inflammatory Eye Disease
The facts most likely to be tested
Scleritis presents with severe, boring ocular pain that frequently radiates to the temple, brow, or jaw and often wakes the patient from sleep.
Physical examination reveals deep, violaceous, or bluish-red scleral injection that does not blanch with topical phenylephrine.
Scleritis is strongly associated with systemic autoimmune diseases, most notably rheumatoid arthritis, granulomatosis with polyangiitis, and relapsing polychondritis.
The painful nature of scleritis and the lack of blanching with phenylephrine are the primary clinical features used to distinguish it from episcleritis.
Anterior scleritis is the most common form, while posterior scleritis may present with vision loss, disc edema, or choroidal folds on funduscopic exam.
Systemic corticosteroids or non-steroidal anti-inflammatory drugs (NSAIDs) are the first-line treatment for non-necrotizing scleritis.
Necrotizing scleritis is a medical emergency that requires urgent systemic immunosuppression to prevent scleral perforation and permanent vision loss.
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A 52-year-old woman with a history of rheumatoid arthritis presents to the clinic complaining of severe, boring left eye pain that has worsened over the past 3 days. She reports that the pain is so intense it wakes her up at night. On physical exam, there is a deep, violaceous injection of the sclera that does not blanch upon the application of 2.5% phenylephrine drops. Her visual acuity is 20/25, and the cornea is clear.
What is the most likely diagnosis?
Scleritis
The patient's presentation of severe, boring pain that wakes her from sleep, combined with the classic finding of non-blanching violaceous scleral injection, is pathognomonic for scleritis, which is highly associated with her underlying rheumatoid arthritis.
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Etiology / Epidemiology
Associated with autoimmune disease in 50% of cases, most commonly rheumatoid arthritis.
Clinical Manifestations
Severe, boring eye pain radiating to the face; blue-hue sclera on exam.
Diagnosis
Clinical diagnosis; slit-lamp examination is the gold standard.
Treatment
Systemic NSAIDs for mild cases; systemic corticosteroids for severe/necrotizing disease.
Prognosis
Risk of scleral perforation and permanent vision loss if untreated.
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Epidemiology & Etiology
Most common in women aged 40-60. Strongly linked to systemic inflammatory conditions, particularly rheumatoid arthritis, Wegener's granulomatosis, and relapsing polychondritis. Infectious causes like herpes zoster ophthalmicus must be excluded.
Pertinent Anatomy
The sclera is a dense, avascular connective tissue layer. Inflammation here is deeper and more destructive than in the superficial episclera. The limbus serves as the transition zone to the cornea.
Pathophysiology
Scleritis is a T-cell mediated immune response causing collagen degradation. Chronic inflammation leads to thinning of the scleral wall. If untreated, the sclera becomes translucent, revealing the underlying uvea.
Clinical Manifestations
Patients present with severe, boring eye pain that often wakes them at night. Physical exam reveals scleral injection that does not blanch with phenylephrine. Red flag: necrotizing scleritis presents with scleral thinning and potential for globe rupture.
Diagnosis
Diagnosis is primarily clinical via slit-lamp examination. The key differentiator from episcleritis is the lack of blanching with topical vasoconstrictors. Ultrasound biomicroscopy may be used to assess the extent of scleral thinning.
Treatment
Initial management for non-necrotizing cases is high-dose oral NSAIDs. If refractory or necrotizing, systemic corticosteroids are required. Contraindications: Topical steroids are ineffective and may mask progression. Immunomodulatory agents like methotrexate are used for steroid-sparing therapy.
Prognosis
Complications include scleral perforation, uveitis, and secondary glaucoma. Patients require frequent ophthalmology follow-up to monitor for thinning and systemic disease progression.
Differential Diagnosis
Episcleritis: Painless or mild discomfort; blanches with phenylephrine
Conjunctivitis: Purulent discharge; diffuse redness
Uveitis: Ciliary flush; cells and flare on slit-lamp
Corneal ulcer: Epithelial defect with fluorescein uptake
Angle-closure glaucoma: Mid-dilated fixed pupil; elevated intraocular pressure