19.3 Uveitis, Scleritis & Orbital Disease
Key Takeaways
- Uveitis is a separately enumerated Ophthalmology blueprint subsection, as are miscellaneous ophthalmologic disorders including episcleritis, scleritis, preseptal cellulitis, endophthalmitis and orbital infections.
- Anterior uveitis presents with a painful photophobic red eye with ciliary flush and cells in the anterior chamber, and is associated with HLA-B27 conditions and sarcoidosis.
- Scleritis produces severe boring pain that wakes the patient and deep violaceous injection that does not blanch with phenylephrine, and often signals systemic vasculitis.
- Orbital cellulitis is distinguished from preseptal cellulitis by proptosis, painful or restricted eye movement and reduced vision, and requires imaging and intravenous antibiotics.
- Endophthalmitis after intraocular surgery or injection presents with pain and rapidly declining vision with a hypopyon and requires emergent intravitreal antibiotics.
1. Uveitis
Inflammation of the uveal tract, classified anatomically because the classification predicts both the symptoms and the associated systemic disease.
| Type | Structures | Symptoms | Findings |
|---|---|---|---|
| Anterior (iritis, iridocyclitis) | Iris, ciliary body | Pain, photophobia, redness, blurred vision, tearing | Ciliary flush (perilimbal injection), cells and flare in the anterior chamber, small or irregular pupil, keratic precipitates, sometimes hypopyon |
| Intermediate | Vitreous, pars plana | Floaters, painless blurring | Vitreous cells and snowballs |
| Posterior | Choroid, retina | Painless vision loss, floaters, scotoma | Chorioretinal lesions, retinal vasculitis |
| Panuveitis | All | Combination | Combination |
Consensual photophobia — pain in the affected eye when light is shone into the unaffected eye — is a useful bedside sign of true intraocular inflammation, because it is produced by consensual pupillary constriction rather than by direct light.
Systemic associations — the reason this is an internal medicine topic
| Pattern | Associations |
|---|---|
| Acute anterior uveitis, unilateral, recurrent | HLA-B27: ankylosing spondylitis, reactive arthritis, psoriatic arthritis, inflammatory bowel disease |
| Granulomatous anterior uveitis | Sarcoidosis, tuberculosis, syphilis |
| Bilateral, chronic | Sarcoidosis, juvenile idiopathic arthritis, Vogt-Koyanagi-Harada disease |
| Posterior uveitis / retinitis | Toxoplasmosis, CMV retinitis in advanced HIV, syphilis, tuberculosis |
| Panuveitis with hypopyon and oral and genital ulcers | Behcet disease |
A first episode of unilateral non-granulomatous anterior uveitis in an otherwise well patient may not require an extensive workup. Recurrent, bilateral, granulomatous or posterior disease does, and the standard evaluation includes syphilis serology, tuberculosis testing, chest imaging and angiotensin-converting enzyme, with HLA-B27 and directed rheumatologic testing as indicated.
Treatment is topical corticosteroid with a cycloplegic agent, prescribed and monitored by ophthalmology. The internist role is prompt referral and evaluation for systemic disease. Never prescribe topical corticosteroids empirically for a red eye — if the cause is herpes simplex keratitis, steroids cause corneal melting and perforation.
2. Episcleritis Versus Scleritis
These sound similar and are managed completely differently, which is why they are a favorite discrimination.
| Episcleritis | Scleritis | |
|---|---|---|
| Pain | Mild irritation or none | Severe, boring, radiating; wakes the patient from sleep |
| Injection | Bright red, superficial, sectoral | Deep violaceous or bluish, diffuse |
| Phenylephrine test | Blanches (vessels are superficial) | Does not blanch |
| Tenderness to palpation | Absent | Present |
| Systemic association | Usually none | Rheumatoid arthritis, granulomatosis with polyangiitis, relapsing polychondritis, lupus, inflammatory bowel disease |
| Vision | Normal | May be reduced |
| Treatment | Self-limited; lubricants, oral NSAIDs | Systemic NSAIDs, corticosteroids, immunosuppression |
Scleritis is a red flag for systemic vasculitis. In a patient with rheumatoid arthritis, scleritis signals severe extra-articular disease, and necrotizing scleritis (scleromalacia perforans) can perforate the globe. New scleritis warrants evaluation for an underlying systemic disease and urgent ophthalmologic referral.
3. Preseptal Versus Orbital Cellulitis
The orbital septum is the anatomic divider, and which side of it the infection sits on determines whether the patient goes home on oral antibiotics or to the operating room.
| Preseptal (periorbital) | Orbital (postseptal) | |
|---|---|---|
| Location | Anterior to the septum | Posterior to the septum |
| Eyelid swelling and erythema | Present | Present |
| Proptosis | Absent | Present |
| Pain with eye movement / ophthalmoplegia | Absent | Present |
| Visual acuity | Normal | May be reduced |
| Afferent pupillary defect | Absent | May be present |
| Fever, toxicity | Variable | Common |
| Source | Skin trauma, insect bite, hordeolum, dacryocystitis | Ethmoid sinusitis in most cases |
| Imaging | Usually not needed | Contrast CT of the orbits and sinuses |
| Treatment | Oral antibiotics, close follow-up | Admission, intravenous antibiotics, surgical drainage of abscess |
The three findings that mean orbital cellulitis are proptosis, painful or restricted extraocular movement, and reduced vision. Any one of them mandates urgent imaging and admission.
Complications of orbital cellulitis include subperiosteal abscess, orbital abscess, cavernous sinus thrombosis (bilateral findings, cranial neuropathies III, IV, V1, V2 and VI, and severe headache — an enumerated blueprint topic under cerebrovascular disease), meningitis and intracranial abscess.
Invasive fungal orbital infection — mucormycosis in a patient with diabetic ketoacidosis or neutropenia, or aspergillosis in the immunocompromised — presents with orbital findings plus a black eschar on the palate or nasal turbinates and is a surgical emergency requiring debridement and amphotericin B.
4. Endophthalmitis
Infection of the intraocular cavities and a true emergency, because vision is lost within hours to days.
Settings:
- Postoperative — most often after cataract surgery, typically within days to weeks
- Post-injection — after intravitreal anti-VEGF therapy
- Post-traumatic — penetrating injury, retained foreign body
- Endogenous — hematogenous seeding in candidemia, bacteremia, endocarditis or injection drug use
Presentation: pain and rapidly decreasing vision with lid swelling, conjunctival injection, hypopyon and loss of the red reflex.
Management is emergent ophthalmologic referral for vitreous sampling and intravitreal antibiotics; systemic antibiotics alone do not achieve adequate intraocular concentrations.
The internal medicine point: in candidemia, ocular involvement changes both the duration and choice of therapy, and patients may be asymptomatic early. A dilated ophthalmologic examination is recommended in candidemia, and any patient with bacteremia or fungemia who reports visual symptoms needs urgent examination.
5. Other Miscellaneous Ophthalmologic Disorders
- Corneal abrasion — pain, foreign body sensation and photophobia with a fluorescein-staining defect. Do not patch, and never prescribe topical anesthetics for home use — they inhibit epithelial healing and mask worsening injury. Contact lens-associated abrasions require antipseudomonal coverage.
- Keratoconjunctivitis sicca (dry eye) — an enumerated topic under conjunctivitis. Burning and foreign-body sensation with paradoxical tearing; consider Sjogren syndrome, medications with anticholinergic effect, and prior radiation.
- Subconjunctival hemorrhage — painless, sharply demarcated blood with normal vision. Benign; consider recurrent episodes as a clue to bleeding diathesis, anticoagulation or, rarely, hypertension.
- Roth spots — an enumerated retinal topic. White-centered retinal hemorrhages seen in endocarditis, leukemia, severe anemia and diabetes.
A 43-year-old woman with seropositive rheumatoid arthritis reports four days of severe, boring left eye pain that wakes her at night, with deep violaceous injection of the sclera. The eye is tender to palpation through the lid, and instillation of topical phenylephrine does not blanch the injected vessels. What is the most appropriate assessment?
A 52-year-old man with poorly controlled diabetes presents with three days of left eyelid swelling and erythema after several days of nasal congestion. Examination shows proptosis, pain with attempted eye movement, and visual acuity reduced to 20/70 on the left. What is the most appropriate management?