UVEITIS

UVEITIS - Teresa A. Everson, MD; William L. Toffler, MD
BASICS
DESCRIPTION
• Uveitis is a nonspecific term used to describe any intraocular inflammatory disorder. Symptoms vary depending on depth of involvement and associated conditions.
- Anterior uveitis: Refers to ocular inflammation limited to the iris (iritis) alone or iris and ciliary body (iridocyclitis)
- Intermediate uveitis: Refers to inflammation of the structures just posterior to the lens (pars planitis or peripheral uveitis)
- Posterior uveitis: Refers to inflammation of the choroid (choroiditis), retina (retinitis), or vitreous near the optic nerve and macula
• System(s) Affected: Nervous
• Synonym(s): Iritis; Iridocyclitis; Choroiditis; Retinochoroiditis; Chorioretinitis; Anterior uveitis; Posterior uveitis; Pars planitis; Panuveitis
ALERT
Geriatric Considerations
The inflammatory response to systemic disease may be suppressed.
Pediatric Considerations
Infection should be the primary consideration. Allergies and psychologic factors (depression, stress) may serve as a triggers. Trauma is also a common cause in this population.
Pregnancy Considerations
May be of importance in the selection of medications
EPIDEMIOLOGY
• Predominant age: All ages
• Predominant sex: Male = Female (except for HLA-B27 anterior uveitis: Male > Female [2.5:1])
Incidence
Anterior uveitis most common (8.2 cases/100,000 annual incidence)
Prevalence
Iritis is 4  more prevalent than posterior uveitis.
RISK FACTORS
No specific risk factors. Higher incidence seen with specific associated conditions
Genetics
• No specific pattern for uveitis in general
• Iritis: Of patients, 50-70% are HLA-B27 positive.
ETIOLOGY
• Infectious: May result from viral, bacterial, parasitic, or fungal etiologies
• Suspected immune-mediated: Possible autoimmune or immune-complex-mediated mechanism postulated in association with systemic (especially rheumatologic) disorders
• Isolated eye disease
• Idiopathic (~25%)
• Masquerade syndromes: Diseases such as malignancies that may be mistaken for primary inflammation of the eye
ASSOCIATED CONDITIONS
• Viral infections: HIV, herpes simplex, herpes zoster, cytomegalovirus
• Bacterial infections: Tuberculosis, leprosy, propionibacterium infection, syphilis, leptospirosis, brucellosis, Lyme disease, Whipple disease
• Parasitic infections: Toxoplasmosis, acanthamebiasis, toxocariasis, cysticercosis, onchocerciasis
• Fungal infections: Histoplasmosis, coccidioidomycosis, candidiasis, aspergillosis, sporotrichosis, blastomycosis, cryptococcosis
• Suspected immune-mediated: Ankylosing spondylitis, Behccet disease, Crohn disease, drug or hypersensitivity reaction, interstitial nephritis, juvenile rheumatoid arthritis, Kawasaki disease, multiple sclerosis, psoriatic arthritis, Reiter syndrome, relapsing polychondritis, sarcoidosis, Sjogren syndrome, systemic lupus erythematosus, ulcerative colitis, vasculitis, vitiligo, Vogt-Koyanagi (Harada) syndrome
• Isolated eye disease: Acute multifocal placoid pigmentary epitheliopathy, acute retinal necrosis, bird-shot choroidopathy, Fuch heterochromatic cyclitis, glaucomatocyclitic crisis, lens-induced uveitis, multifocal choroiditis, pars planitis, serpiginous choroiditis, sympathetic ophthalmia, trauma
• Masquerade syndromes: Leukemia, lymphoma, retinitis pigmentosa, retinoblastoma

DIAGNOSIS
SIGNS AND SYMPTOMS
• Anterior uveitis (~80% of patients with uveitis)
- Decreased visual acuity
- Generally acute in onset
- Deep eye pain
- Photophobia (consensual)
- Conjunctival vessel dilation
- Perilimbal (circumcorneal) dilation of episcleral and scleral vessels (ciliary flush)
- Small pupillary size of affected eye
- Hypopyon or hyphema (white or red blood cells pooled in the anterior chamber)
- Frequently unilateral (95% of HLA-B27-associated cases)
- Bilateral involvement and systemic symptoms (fever, fatigue, abdominal pain) may be associated with interstitial nephritis.
- Systemic disease is most likely to be associated with anterior uveitis (in 1 study, 53% of patients found to have systemic disease).
• Intermediate and posterior uveitis
- Decreased visual acuity
- Unresolving floaters
- Generally insidious in onset
- More commonly bilateral
- Posterior inflammation will generally cause minimal pain or redness unless associated with an iritis.
Physical Exam
Slit-lamp examination and indirect ophthalmoscopy are necessary for precise diagnosis.
TESTS
Lab
• No specific test for the diagnosis of uveitis. Tests for etiologic factors or associated conditions should be based on history and physical examination.
• CBC, BUN, creatinine (interstitial nephritis)
• HLA-B27 typing (ankylosing spondylitis, Reiter syndrome)
• Antinuclear antibody, ESR (systemic lupus erythematosus, Sjogren syndrome)
• Venereal Disease Research Lab test, fluorescent titer antibody (syphilis)
• Purified protein derivative (PPD) tuberculin skin test (tuberculosis)
• Lyme serology (Lyme disease)
• Special tests
• Disorders that may alter lab results: Immune deficiency
Imaging
• Chest x-ray (sarcoidosis, histoplasmosis, tuberculosis, lymphoma)
• Sacroiliac x-ray (ankylosing spondylitis)
Diagnostic Procedures/Surgery
Slit-lamp examination
Pathological Findings
Keratic precipitates, inflammatory cells in anterior chamber or vitreous, synechiae (fibrous tissue scarring between iris and lens), macular edema, perivasculitis of retinal vessels
DIFFERENTIAL DIAGNOSIS
• Conjunctivitis
• Episcleritis
• Scleritis
• Keratitis
• Acute angle-closure glaucoma
TREATMENT
GENERAL MEASURES
• Appropriate health care: Outpatient with urgent ophthalmologic consultation
• Medical therapy best initiated following full ophthalmologic evaluation
• Treatment of underlying cause, if identified
• Cycloplegia
• Anti-inflammatory therapy
Diet
No special diet
Activity
Full activity
MEDICATION (DRUGS)
First Line
• Caution should be used when using empiric treatment; referral to an ophthalmologist is recommended in most cases.
• Homatropine hydrobromide (Isopto) 2% ophthalmic solution: 2 drops to the affected eye b.i.d., or as often as q3h if necessary; plus
• Prednisolone acetate 1% ophthalmic suspension: 2 drops to the affected eye q1h initially, tapering to once a day with improvement
• Contraindications
- Hypersensitivity to the medication or component of the preparation
- Cycloplegia is contraindicated in patients known to have, or be predisposed to, glaucoma.
- Topical corticosteroid therapy is contraindicated in uveitis secondary to infectious etiologies.
• Precautions
- Homatropine hydrobromide may produce adverse systemic antimuscarinic effects. Use extreme caution in infants and young children because of increased susceptibility to systemic effects.
- Topical corticosteroids may increase intraocular pressure. Prolonged use may cause cataract formation and exacerbate existing herpetic keratitis which may masquerade as iritis.
• Significant possible interactions
- Refer to manufacturer's profile for each drug.
Second Line
• Cycloplegia: Scopolamine hydrobromide 0.25% (Isopto Hyoscine) up to t.i.d. or cyclopentolate hydrochloride 1% (Cyclogyl)
• Anti-inflammatory: Prednisolone sodium phosphate 1% (Ocu-Pred Forte), dexamethasone sodium phosphate 0.1% (Ocu-Dex), and dexamethasone suspension
• Systemic NSAIDs may provide some benefit.
FOLLOW-UP
PROGNOSIS
• Dependent on the presence of causal diseases, or associated conditions
• Uveitis resulting from infections (systemic or local) tend to resolve with eradication of the underlying infection.
• Uveitis associated with seronegative arthropathies tend to be acute (lasting 3 months) and frequently recurrent.
COMPLICATIONS
Loss of vision as a result of the following
• Keratic precipitate deposition on the corneal or lens surfaces
• Increased intraocular pressure, acute angle-closure glaucoma
• Formation of synechiae
• Cataract formation
• Vasculitis with vascular occlusion, retinal infarction
• Macular edema
• Optic nerve damage
PATIENT MONITORING
• Ophthalmologic follow-up as recommended by consultant
• Schedule for complete history and physical to evaluate for associated systemic disease.
REFERENCES
1. McCluskey PJ, Towler HM, Lightman S. Management of chronic uveitis. BMJ. 2000;320(7234):555-558.
2. Schiffman RM, Jacobsen G, Whitcup SM. Visual functioning and general health status in patients with uveitis. Arch Ophthalmol. 2001;119(6):841-849.
3. Smith JR, Rosenbaum JT. Management of uveitis: A rheumatologic perspective. Arthritis Rheum. 2002;46(2):309-318.
4. Patel H, Goldstein D. Pediatric uveitis. Pediatr Clin North Am. 2003;50(1):125-136.
5. Lee AG, Beaver HA. Painful ophthalmologic disorders and eye pain for the neurologist. Neurol Clin. 2004;22(1):75-97.
MISCELLANEOUS
• See also: Conjunctivitis, acute; Glaucoma, primary angle-closure; Scleritis
• Other notes
- Synonyms are anatomic descriptions of the focus of the uveal inflammation.
- Severe or unresponsive uveitis may require therapy including periocular injection of corticosteroids, systemic corticosteroids, cytotoxic agents (azathioprine, cyclophosphamide, chlorambucil, and methotrexate), immunosuppressive agents (cyclosporine), immunomodulatory agents (sulfasalazine), or tumor necrosis factor inhibitors (infliximab, etanercept).