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Overview of Uveitis

by Emmett T. Cunningham, Jr., MD, PhD, MPH

Uveitis is defined as inflammation of the uveal tract—the iris, ciliary body, and choroid. However, the retina and fluid within the anterior chamber and vitreous are often involved as well. Most cases are idiopathic and presumed autoimmune; identifiable causes include trauma, infection, and systemic diseases, many of which are autoimmune. Symptoms include decreased vision, ocular ache, redness, photophobia, and floaters. Although uveitis is identified clinically, identifying the cause typically requires testing. Treatment depends on cause but typically includes topical, locally injected, or systemic corticosteroids with a topical cycloplegic-mydriatic drug. Noncorticosteroid immunosuppressive drugs may be used in severe and refractory cases.

Uveitis is classified anatomically as

  • Anterior uveitis: Localized primarily to the anterior segment of the eye, includes iritis (inflammation in the anterior chamber alone) and iridocyclitis (inflammation in the anterior chamber and anterior vitreous)

  • Intermediate uveitis (peripheral uveitis or chronic cyclitis): Localized to the vitreous

  • Posterior uveitis: Any form of retinitis, choroiditis, or inflammation of the optic disk

  • Panuveitis (also called diffuse uveitis): Inflammation in both the anterior and posterior chambers

Uveitis may occur with or without vitreitis, retinitis, papillitis, or optic neuritis.

Etiology

Causes of anterior uveitis are

  • Idiopathic or post-surgical (most common cause)

  • Trauma (probably the next most common cause)

  • Spondyloarthropathies

  • Juvenile idiopathic arthritis

  • Herpesvirus (herpes simplex and varicella-zoster) infection

Causes of intermediate uveitis are

  • Idiopathic (most common)

  • Multiple sclerosis

  • Sarcoidosis

  • TB

  • Syphilis

  • Lyme disease (in endemic regions)

The latter five in this list are uncommon causes of intermediate uveitis.

Causes of posterior uveitis (retinitis) are

  • Idiopathic (most common)

  • Toxoplasmosis, (in immunocompetent patients)

  • CMV (in patients with HIV/AIDS)

Causes of panuveitis are

  • Idiopathic (most common)

  • Sarcoidosis

Infrequently, systemic drugs cause uveitis (usually anterior). Examples are sulfonamides, pamidronate (an inhibitor of bone resorption), rifabutin, and cidofovir.

Systemic diseases causing uveitis and their treatment are discussed elsewhere in T he M ANUAL .

Symptoms and Signs

Symptoms and signs may be subtle and vary depending on the site and severity of inflammation.

Anterior uveitis tends to be the most symptomatic, usually manifesting with

  • Pain (ocular ache)

  • Redness

  • Photophobia

  • Decreased vision (to a variable degree)

Signs include hyperemia of the conjunctiva adjacent to the cornea (ciliary flush or limbal injection). Slit-lamp findings include keratic precipitates (WBC clumps on the inner corneal surface), cells and flare (a haze) in the anterior chamber (aqueous humor), and posterior synechiae. With severe anterior uveitis, WBCs may layer in the anterior chamber (hypopyon).

Intermediate uveitis is typically painless and manifests with

  • Floaters

  • Decreased vision

The primary sign is cells in the vitreous humor. Aggregates and condensations of inflammatory cells often occur over the pars plana (near the junction of the iris and sclera), forming "snowballs." Vision may be decreased because of floaters or cystoid macular edema, which results from fluid leakage from blood vessels in the macula. Confluent and condensed vitreous cells and snowballs over the pars plana may cause a classic "snowbank" appearance, which can be associated with neovascularization of the retinal periphery.

Posterior uveitis may give rise to diverse symptoms but most commonly causes floaters and decreased vision as occurs in intermediate uveitis. Signs include

  • Cells in the vitreous humor

  • White or yellow-white lesions in the retina (retinitis), underlying choroid (choroiditis), or both

  • Exudative retinal detachments

  • Retinal vasculitis

  • Optic disk edema

Panuveitis may cause any combination of the previously mentioned symptoms and signs.

Complications

Serious complications of uveitis include profound and irreversible vision loss, especially when uveitis is unrecognized, inadequately treated, or both.

The most frequent complications include

  • Cataract

  • Glaucoma

  • Retinal detachment

  • Neovascularization of the retina, optic nerve, or iris

  • Cystoid macular edema (the most common cause of decreased vision in patients with uveitis)


Diagnosis

  • Slit-lamp examination

  • Ophthalmoscopy after pupil dilation

Uveitis should be suspected in any patient who has ocular ache, redness, photophobia, floaters, or decreased vision. Patients with anterior uveitis have ocular ache in the affected eye if light is shined in the unaffected eye (true photophobia), which is uncommon in conjunctivitis.

Diagnosis of anterior uveitis is by recognizing cells and flare in the anterior chamber. Cells and flare are seen with a slit lamp and are most evident when using a narrow, intensely bright light focused on the anterior chamber in a dark room. Findings of intermediate and posterior uveitis are most easily seen after dilating the pupil (see Ophthalmoscopy). Indirect ophthalmoscopy (usually done by an ophthalmologist) is more sensitive than direct ophthalmoscopy. (N ote : If uveitis is suspected, patients should be referred immediately for complete ophthalmologic evaluation.)

Many conditions that cause intraocular inflammation can mimic uveitis and should be considered in the appropriate clinical settings. Such conditions include, most commonly, severe conjunctivitis (eg, epidemic keratoconjunctivitis), severe keratitis (eg, herpetic keratoconjunctivitis, peripheral ulcerative keratitis), and severe scleritis, and, less commonly, intraocular cancers in the very young (typically retinoblastoma and leukemia) and in the elderly (intraocular lymphoma). Much less commonly, retinitis pigmentosa (see Retinitis Pigmentosa) can manifest with mild inflammation, which may be confused with uveitis.

Treatment

  • Corticosteroids (usually topical)

  • Cycloplegic-mydriatic drugs

Treatment of active inflammation usually involves corticosteroids given topically (eg, prednisolone acetate 1% 1 drop q 1 h while awake) or by periocular or intraocular injection along with a cycloplegic-mydriatic drug (eg, homatropine 2% or 5% drops [if available] or cyclopentolate 0.5% or 1.0% drops, either drug given bid to qid depending on severity). Antimicrobial drugs are used to treat infectious uveitis. Particularly severe or chronic cases may require systemic corticosteroids (eg, prednisone 1 to 2 mg/kg po once/day), systemic noncorticosteroid immunosuppressive drugs (eg, methotrexate 15 to 20 mg po once/wk), laser phototherapy, cryotherapy applied transsclerally to the retinal periphery, or surgical removal of the vitreous (vitrectomy).

Key Points

  • Inflammation of the uveal tract (uveitis) can affect the anterior segment (including the iris), intermediate uveal tract (including the vitreous), or posterior uvea (including the choroid, retina, and optic nerve).

  • Most cases are idiopathic, but known causes include infections, trauma, and autoimmune disorders.

  • Findings in anterior uveitis include aching eye pain, photophobia, redness closely surrounding the cornea (ciliary flush), and, on slit-lamp examination, cells and flare.

  • Intermediate and posterior uveitis tend to cause less pain and eye redness but more floaters and decreased vision.

  • Diagnosis is confirmed by slit-lamp examination and ophthalmoscopic examination (often indirect) after pupillary dilation.

  • Treat in conjunction with an ophthalmologist, usually with topical corticosteroids and cycloplegic-mydriatic drugs.

Resources In This Article

Drugs Mentioned In This Article

  • Drug Name
    Select Trade
  • VISTIDE
  • MYCOBUTIN
  • AREDIA
  • TUSSIGON
  • OTREXUP
  • ORAPRED, PRELONE
  • RAYOS
  • AKPENTOLATE, CYCLOGYL

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