BASICS

DESCRIPTION

Acute anterior uveitis (AAU) involves inflammation of the iris alone (iritis) or iris and ciliary body together (iridocyclitis) of abrupt onset (lasting days to weeks) and limited duration (<3 months).

EPIDEMIOLOGY

Incidence

• 17 cases per 100,000 people in developed countries

• Varying incidence in the general populations depending on the population and country of residence

Prevalence

38 cases per 100,000 people in developed countries

RISK FACTORS

Genetics

HLA-B27 phenotype is a risk factor, seen in 50% of acute anterior uveitis patients in a Caucasian population.

PATHOPHYSIOLOGY

Intraocular inflammation of the iris or iris and ciliary body usually causes pain, redness, blurred vision, and photophobia.

ETIOLOGY

Numerous etiologies can trigger acute anterior uveitis, including: Infections, autoimmune diseases, trauma, surgery, medications, and lens-related disorders. Approximately one-third to half of the cases are idiopathic.

COMMONLY ASSOCIATED CONDITIONS

A number of systemic diseases can be associated with acute anterior uveitis, including HLA-27 associated diseases (Ankylosing spondylitis, inflammatory bowel disease, reactive arthritis, psoriatic arthritis), sarcoidosis, syphilis, Lyme disease, HIV, tuberculosis, collagen vascular diseases, and juvenile idiopathic arthritis.

DIAGNOSIS

HISTORY

• Classic symptoms are pain, redness, photophobia, and blurred vision.

• Detailed review of symptoms and past medical history should be taken with special attention to rheumatologic, dermatologic, gastrointestinal, and autoimmune diseases. Infectious exposures, sexual history, medication usage, and family history (with attention to rheumatologic and inflammatory diseases) should also be included. Ocular history is critical to assess onset and progression of symptoms, history of prior episodes, and success of prior treatments.

PHYSICAL EXAM

• Ciliary flush (perilimbal dilated episcleral vessels are caused by increased blood flow to the ciliary body) is often present.

• Pupillary miosis may be seen.

• Keratic precipitates (KPs) on the corneal endothelium may be fine (non-granulomatous KP) or large and have a “mutton fat” appearance (granulomatous KP).

• Anterior chamber exam may show cell, flare, and rarely, hypopyon (HLA-B27-related and Behçet disease in particular). Iris nodules may be present at the pupillary margin or iris stroma.

• Iris ischemia can cause iris stromal atrophy and may indicate previous episodes.

• Posterior synechiae, which are adhesions between the posterior iris and anterior lens capsule, may be seen. Gonioscopy can reveal peripheral anterior synechiae.

• The intraocular pressure may be high or low.

– Dilated fundus examination should be performed to look for vitreitis, snowballs, snow banks, optic disc edema and hyperemia, vessel sheathing, perivascular exudates, retinitis, and choroidal infiltrates. Though cystoid macular edema and optic disc edema may be seen in acute anterior uveitis, other posterior segment involvement constitutes panuveitis and changes the differential diagnosis.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• To evaluate patients for an underlying systemic disease, lab testing is performed for acute anterior uveitis that is one of the following: Severe, recurrent, granulomatous, or bilateral

– History and examination should guide the selection of lab tests, which may initially include CBC, ESR, PPD, FTA-ABS and VDRL, Lyme antibodies, HLA-B27, ANA, ACE, and HIV testing. Numerous other tests can be helpful as the clinical picture dictates.

Imaging

• Chest x-ray is done to look for pulmonary signs of tuberculosis or sarcoidosis.

– In cases of suspected sarcoidosis, chest computed tomography (CT) scan has higher sensitivity and specificity than chest x-ray.

DIFFERENTIAL DIAGNOSIS

• The causes of AAU is broad, but the most common causes are:

• Idiopathic (approximately half of all cases of AAU)

• HLA-B27 associated uveitis (ocular disease alone or associated with a systemic syndrome such as reactive arthritis, psoriatic arthritis, ankylosing spondylitis, or inflammatory bowel disease)

• Infectious causes (viral [HSV, VZV, CMV, HIV], bacterial [syphilis, TB, Lyme], fungal, and parasitic)

• Sarcoidosis

• Behçet’s disease

• Systemic lupus erythematosus

• Juvenile idiopathic arthritis-related uveitis

• Trauma-related iritis

• Lens-induced uveitis

• Fuchs heterochromic iridocyclitis

• Posner-Schlossman syndrome

• Leukemia, lymphoma (masquerade syndromes)

• Idiopathic

TREATMENT

MEDICATION

First Line

• Corticosteroids are the drug of choice and are dosed based on disease severity

• Topical corticosteroids are easily administered with the least side effects (e.g., prednisolone 1% or Durezol (difluprednate), 1 gtt q1–6 h

• Periocular and/or systemic corticosteroids are indicated for disease unresponsive to topical medication

• Mydriatics/cycloplegics are added to relieve ciliary muscle spasm, prevent posterior synechiae, and help stabilize the blood-aqueous barrier (e.g., cyclopentolate 0.5–2% 1 gtt t.i.d, or atropine 1% or homatropine b.i.d.)

Second Line

• Immunosuppressive agents (e.g., methotrexate, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus) and biologics (infliximab, adalimumab) are used in corticosteroid-resistant cases or as steroid-sparing agents.

– The fluocinolone implant (Retisert) or dexamethasone implant (Ozurdex) may be very helpful, but may be associated with an increased rate of cataract or ocular hypertension.

ADDITIONAL TREATMENT

Issues for Referral

Immunosuppressive agents are started in conjunction with an internist/rheumatologist, or uveitis specialist.

Additional Therapies

Elevated intraocular pressure can be treated with topical beta blockers, carbonic anhydrase inhibitors, alpha agonists, or hyperosmolar agents. Prostaglandins should be avoided as they may be proinflammatory. Topical NSAIDs can be used to treat cystoids macular edema.

SURGERY/OTHER PROCEDURES

• Cataract extraction may be done in an eye that is quiet for at least 3 months prior to surgery.

• Glaucoma surgery may be needed for intraocular pressures not responsive to topical therapy.

• Pre- and postoperative systemic and topical steroids are given in the perioperative period to prevent recurrence of inflammation.

IN-PATIENT CONSIDERATIONS

Admission Criteria

Patients with severe vision or life-threatening disease may need to be admitted to provide aggressive immunosuppressive therapy.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patients require close monitoring until the inflammation is controlled. Then, frequency of medication and follow-up visits may slowly be reduced.

Patient Monitoring

• Patients require measurement of visual acuity and intraocular inflammation at each visit.

• Careful grading of cell and flare at initial and follow-up visits can be used to assess response to treatment.

COMPLICATIONS

Cataract formation may be caused by inflammation and corticosteroids. Glaucoma may be caused by trabeculitis, peripheral anterior synechiae, and iris neovascularization. Hypotony can result from ciliary body shutdown or cyclitic membranes. Cystoid macular edema may also occur.

ADDITIONAL READING

• Agrawal RV, Murthy S, Sangwan V, et al. Indian J Ophthalmol 2010;58(1):11–19.

• Weinberg RS. Anterior Uveitis. Ophthalol Clin N Amer 1993;6(1):23–28.

• Rothova A, van Veenendaal WG, Linssen A, et al. Clinical feature of acute anterior uveitis. Amer J Ophthalmol 1987;103:137–145.

• Pavesio CE, Nozik RA. Anterior and intermediate uveitis. Int Ophthalmol Clin 1990;30(4):244–251.

CODES

ICD9

• 364.00 Acute and subacute iridocyclitis, unspecified

• 364.3 Unspecified iridocyclitis

CLINICAL PEARLS

• Classic findings are abrupt onset of pain, redness, blurred vision, and photophobia accompanied by cell and flare in the anterior chamber.

• Targeted work-up can reveal an underlying systemic condition.

• Topical, periocular, and systemic corticosteroids, as well as steroid-sparing agents, are used as needed to control all intraocular inflammation whenever possible.

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