BASICS

DESCRIPTION

• Uveitis: intraocular inflammation

– Anterior: involving iris and ciliary body (e.g., iritis, iridocyclitis)

– Intermediate: involving pars plana (i.e., pars planitis)

– Posterior: involving retina (i.e., retinitis) and choroid (i.e., choroiditis), and, possibly, the optic nerve (papillitis or optic neuritis) and vitreous (vitreitis)

– Panuveitis: 2 or more of the above

EPIDEMIOLOGY

Incidence

4.3–6.9 per 100,000

Prevalence

• 30 cases per 100,000 (1)[A]

• Uveitis is much less common in children than in adults

RISK FACTORS

• Genetic predisposition (HLA-B27)

– Juvenile idiopathic arthritis (JIA) is the most common cause other than idiopathic causes and trauma

– Pauciarticular disease

– Age <7 years at presentation

– ANA positive, RF negative

– Female gender

• Other autoimmune diseases

• Pre- or postnatal infection (e.g., toxoplasmosis, toxocariasis, tuberculosis)

• See Etiology

Genetics

• HLA-B27

– Associated with anterior uveitis, ankylosing spondylitis, Reiter syndrome, inflammatory bowel disease, and psoriatic arthritis

• Genetic predisposition may be coupled with environmental trigger to produce disease

GENERAL PREVENTION

• Appropriate monitoring of systemic disease and periodic ophthalmologic examination of those at risk

• Avoidance of infection

PATHOPHYSIOLOGY

• Dependent upon etiology

• JIA: Pathophysiology unknown

ETIOLOGY

• Anterior

– Juvenile idiopathic arthritis (JIA)

– Most common cause of noninfectious anterior uveitis

– Oligoarticular: greatest risk of uveitis

– Systemic: least risk of uveitis

– Eye involvement may precede joint activity

– No correlation between eye and joint disease

– Onset and activity is asymptomatic with white, quiet eye

– ∼50% may require long-term treatment

– Enthesis-related (HLA-B27): ankylosing spondylitis, Reiter syndrome, inflammatory bowel disease, psoriatic arthritis

– Idiopathic

– Trauma

– Behçet’s disease

– Sarcoidosis

– Kawasaki disease

– Tubulointerstitial nephritis and uveitis (TINU)

– Masquerade syndromes:

– Retinoblastoma

– Lymphoma or leukemia

– Trauma and/or intraocular foreign body

– Juvenile xanthogranuloma (JXG)

– Coat’s disease

– Infectious

Herpes simplex or varicella zoster virus

Syphilis, tuberculosis, Lyme’s disease

Parasitic infection (e.g., toxoplasmosis)

• Intermediate

– Idiopathic

– Sarcoidosis

– Inflammatory bowel disease

– Multiple sclerosis

– Lyme’s disease

– Uncommon in JIA

• Posterior

– Infectious

– Toxoplasmosis: most common

– Less frequent: toxocariasis, tuberculosis, syphilis, Lyme’s disease, cat scratch disease, parasitic, fungal, bacterial, and viral including HSV, varicella, and cytomegalovirus

– Noninfectious

Masquerade syndrome

Sarcoidosis

Sympathetic ophthalmia

Vogt–Koyanagi–Harada disease

• Panuveitis

– Infection

– Systemic disease

– Masquerade syndrome

COMMONLY ASSOCIATED CONDITIONS

Cataract, glaucoma, band keratopathy, posterior synechiae, chorioretinal scar, exudative retinal detachment, hypotony, and visual loss

DIAGNOSIS

HISTORY

• May be key to determining diagnosis

• Ocular symptoms (NOTE: absence of symptoms does not rule out uveitis, especially in JIA)

• Medical illnesses

• Review of systems, family history, sexual history, and travel history

PHYSICAL EXAM

• General physical exam

• Complete ophthalmologic examination

– Visual acuity testing

– Intraocular pressure

– Conjunctiva

– Ciliary flush, nodules, episcleritis, and scleritis

– Cornea

– Edema from inflammation or glaucoma

– Keratitic precipitates: collections of inflammatory cells deposited on endothelium; small-nongranulomatous; larger with greasy appearance—granulomatous (Mutton Fat)

– Band keratopathy

– Dendrite or geographic ulcer with HSV

– Interstitial keratitis

– Anterior chamber

– Cells measured +1 to +4 or cells/slit-lamp field

– Flare: leakage of protein

– Hypopyon: white blood cells settled in lower anterior chamber

– Pseudohypopyon: tumor cells

– Iris

– Posterior synechiae: adhesion between iris and lens capsule

– Peripheral anterior synechiae: adhesion between iris and peripheral cornea

– neovascularization

– Lens: cataract

– Gonioscopy

– Vitreous cells, blood, or membranes

– Retina

– Cystoid macular edema, cotton wool spots, hemorrhage, vascular sheathing, neovascularization, or detachment

– Choroid

– Dalen–Fuchs nodules: yellowish lesions with hyperpigmentation seen with sarcoid and sympathetic ophthalmia

– Pars plana

– Snowbanking: white masses

– Neovascularization

– Optic nerve: swelling, atrophy, neovascularization, and infiltration

DIAGNOSTIC TESTS & INTERPRETATION

Directed work up based on history and physical findings

Lab

• Blood work if no clear cause

– ACE, ANA, calcium, CBC with differential, ESR, Lyme titer (if geographic risk), RF, VDRL/FTA-ABS

• Urinalysis

Imaging

• CXR

• Ocular ultrasound if no view

Diagnostic Procedures/Other

• Hand and SI joint radiographs

• CT/MRI brain and sinuses

• High-frequency ultrasonographic biomicroscopy

• Lumbar puncture

• ANCA, HLAB27, other antibodies or titers, T-cell counts, or HIV

• Stool for ova and parasites

• Skin testing: allergy, anergy, and PPD

• Additional ophthalmic testing

– ERG, VEP

– Fluorescein angiography

– OCT

– Visual field testing

• Biopsy: conjunctiva, lacrimal gland, aqueous, vitreous, or retina

Pathological Findings

Dependent upon etiologic agent

DIFFERENTIAL DIAGNOSIS

• Retinoblastoma

• Leukemia or lymphoma

• Trauma

• Intraocular foreign body

• Retinitis pigmentosa

• Coat’s disease

• Juvenile xanthogranuloma

TREATMENT

MEDICATION

First Line

• Topical steroid: 1 drop in affected eye ranging from q.i.d to every 15 min, based on initial severity and taper based on clinical picture

• Cycloplegic agent to prevent synechiae and for pain control

Second Line

• Periocular injection of steroid

• Systemic therapy should be prescribed and managed by physician familiar with therapies and monitoring for complications (e.g., rheumatologist).

• Least toxic systemic therapy should be instituted when topical therapy is insufficient.

• Systemic steroid (e.g., oral, intravenous)

• Nonsteroidal anti-inflammatory drugs: topical or systemic have a minor role

• Nonsteroidal immunosuppressives

– Antimetabolites

– Methotrexate (for JIA first line, if failing topical steroids)

– Mycophenolate mofetil

– Anti-tumor necrosis factor antibody medications (second line for JIA)

– Infliximab, etanercept, adalimumab

– B-cell-directed therapy: Rituximab

– Immunosuppressives: Cyclosporine, tacrolimus, or azathioprine

– Alkylating agents: Cyclophosphamide, chlorambucil, etc.

ADDITIONAL TREATMENT

General Measures

Based on underlying disease and disability when present

Issues for Referral

• Rheumatology evaluation in all patients without obvious etiology

• Infectious disease, oncology, and other specialties as indicated

Additional Therapies

• Low-vision services

• Counseling for adjustment to vision loss and living with chronic disease

COMPLEMENTARY & ALTERNATIVE THERAPIES

None known to be helpful

SURGERY/OTHER PROCEDURES

• Chelation for band keratopathy

• Cataract surgery

• Glaucoma surgery

• Retinal laser, detachment repair

• Drug-delivery implant

IN-PATIENT CONSIDERATIONS

Admission Criteria

Only required if systemic disease warrants or treatment with short-term intravenous pulse steroids

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Frequent eye examination to address ocular complications of disease

• Control systemic disease

• Monitor systemic treatment

Patient Monitoring

• Appropriate testing based on therapy

• Medication doses based on weight periodically need to be adjusted as child grows.

DIET

No typical dietary restrictions

PATIENT EDUCATION

• www.aapos.org/faq_list/iritis

• www.uveitis.org/

• www.mdjunction.com/arthritis-juvenile-rheumatoid

• www.pgcfa.org

PROGNOSIS

• Early and aggressive therapy can often prevent or delay vision loss.

• Visual disability can significantly impact quality of life (2)[A].

COMPLICATIONS

• Band keratopathy

• Cataract

• Posterior synechiae

• Glaucoma

• Cystoid macular edema (CME)

• Hypotony

• Disc edema

• Vitreous hemorrhage

• Vitreoretinal membrane

• Phthisis

• Permanent vision loss

– May occur in up to 1/3 of uveitis patients

– Posterior uveitis, CME, and hypotony associated with significant vision loss (3)[A]

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