Diseases of the Uvea

Uveitis (General)

• inflammation of the uvea tract

• responsible for 10-15% of total blindness cases

• Onset: varies with peak incidence between 20-59 years of age

  • incidence increases with age

• In adult patients, female are affected more than males

• In pediatric patients, males are affected more than females

• Multiple Causes:

  • Idiopathic

  • Non-infectious or immunologic: with and without known systemic association

  • Infectious: caused by bacteria, virus, fungus, parasite or other infectious agents

  • Traumatic: includes surgery

  • Masquerade: neoplastic or non-neoplastic conditions

Uveitis Classifcations

• Primary site of inflammation:

  • Anterior uveitis: inflammation of the anterior chamber (most common)

  • Intermediate uveitis: inflammation of the vitreous (least common)

  • Posterior uveitis: inflammation of the retina and/or choroid

  • Panuveitis: inflammation of all uveal structures

• Onset:

  • Sudden: characterized by pain, redness and photophobia

  • Insidious: characterized as white and painless

• Duration:

  • Limited: less than or equal to 3 months

  • Persistent: >3 months

• Clinical Course:

  • Acute: sudden onset and limited duration

  • Chronic: persistent uveitis with relapse in less than 3 months after discontinuing tx

  • Recurrent: characterized by repeated episodes separated by periods of inactivity without tx greater than or equal to 3mon long

  • Remission: no visible cells (inactivity) for 3 months or longer

• Histopathology:

  • Non-granulomatous (more common)

  • Granulomatous

• Laterality:

  • Unilateral is more common than bilateral

  • Bilateral is more common with systemic non-infectious conditions

Anterior Uveitis General Symptoms

• Pain: w/ acute inflammation of iris & ciliary body

  • muscles spasm = dull, achy, throbbing, radiating

  • worse when looking at light or up close

• Photophobia: caused by ciliary muscle spasm

Anterior Uveitis General Signs

• Conjunctival injection/circumlimbal injection: enlargement of episcleral vessels around limbus

  • diffuse redness can accompany it

  • dark red

• Keratic precipitates: inflammatory deposits on the endothelium

  • usually in inferior endothelium as triangle = Arlt triangle

    • d/t convection current

  • Fine dusting keratic precipitates: made up of neutrophils and lymphoplasmacytic cells

    • small and white

    • Seen with non-granulomatous uveitis

  • Granulomatous keratic precipitates: made up of lymphocytes, macrophages and epithelioid cells

    • larger, 1mm

    • greasy appearance

    • mutton fat

    • Seen with granulomatous uveitis

• Aqueous cells: mostly made up of lymphocytes

  • large collection in ant. chamber = hypopyon

• Anterior chamber flare: result of the breakdown of the blood aqueous barrier; protein leaks into ant. chamber

• Iris nodules: accumulation of epithelioid cells and lymphocytes

  • Koeppe: located on the pupillary margin (non- and granulomatous)

  • Busacca: located in the iris stroma (granulomatous)

  • Berlin: located in the anterior chamber (granulomatous)

• Anterior synechia: adhesions in the iridocorneal angle

  • can lead to angle closure

• Posterior synechia: adhesions between the iris and the anterior capsule of the lens

  • Seclusio pupilla: a posterior synechia 360°; leads to ris bombé and angle closure

• Intraocular pressure (elevation or reduction)

  • Elevation = blockage of TM by inflammatory cells, peripheral anterior synechia, posterior synechia w/ iris bombé, inc aqeuous viscosity

    • common in chronic uveitis

  • Reduction: due to reduced aqueous production due to ciliary body inflammation & inc aqueous outflow

    • common in acute

• Pupil miosis: due to iris sphincter spasm

  • Increases the risk for a posterior synechia

Hypopyon

inflammatory cells and fibrin that have settled in the inferior portion of anterior chamber; Commonly seen with HLA-B27 uveitis and Behçet’s uveitis

Anterior Uveitis General Complications

• band keratopathy

• glaucoma

• cataracts

• Cystoid macular edema (from chronic inflammation)

Anterior Uveitis General Treatment

• Topical corticosteroid

• Topical cycloplegic: helps with pain management and prevents the formation of a posterior synechia

Causes of anterior uveitis

• Idiopathic (most common)

• Non-infectious or immunologic (2nd most common)

  • Seronegative HLA-B27 associated arthropathies are the most common cause

    • Ankylosing spondylitis, reactive arthritis, inflammatory bowel disease

• Infectious

  • Herpetic keratouveitis infections are the most common

• Masquerade

• Trauma

Characteristics of Seronegative Spondylarthropathies

• Individuals will be rheumatoid factor (RF) and antinuclear antibody (ANA) negative

• Individuals will have a strong HLA-B27 positive association

• Anterior uveitis is a common ocular manifestation

Ankylosing spondylitis

• males > females

  • caucasian

• Onset: teens to early adulthood

• 25% develop anterior uveitis

  • ANY laterality, unilateral most common

  • recurrent

  • non-granulomatous

• Signs: 

  • Circumlimbal hyperemia

  • Cells and flare in the anterior chamber with hypopyon development

  • Fine white/grey keratic precipitates

• Work Up:

  • Imaging of the lower back (MRI or X-ray)

  • HLA-B27

• Complications:

  • Secondary glaucoma

  • Cataract

  • Maculopathy in prolonged or severe cases

• Treatment:

  • Topical cycloplegic

  • Topical corticosteroids

• Other ocular manifestations: conjunctivitis and ssc

Reactive arthritis

• Onset: 18-40 years of age

• males > females

• Classic triad: Arthritis, urethritis, conjunctivitis

  • 3-12% of individuals will have anterior uveitis

    • acute

    • unilateral

    • non-granulomatous

• Symptoms: pain, photophobia

• Presentation:

  • Circumlimbal hyperemia

  • Fine to medium white keratic precipitates

  • MORE cells and flare in the anterior chamber

    • Hypopyon may develop in severe cases

• Complications:

  • Secondary glaucoma

  • Maculopathy

• Treatment:

  • Topical cycloplegic

  • Topical corticosteroids

• Other ocular manifestations: Conjunctivitis, episcleritis, scleritis

Psoriatic arthritis

• Onset: 30 to 40 year olds

• females > males

• Most common ocular manifestation is anterior uveitis

  • acute

  • non-granulomatous

• Signs: pain, photophobia

• Presentation:

  • Circumlimbal hyperemia

  • Fine white keratic precipitates

  • Cells and flare in the anterior chamber

    • Hypopyon occasionally occurs

• Complications:

  • secondary glaucoma

  • maculopathy

• Treatment:

  • topical cycloplegic

  • topical corticosteroids

• Other ocular manifestations: Conjunctivitis, episcleritis, scleritis

Enteropathic Arthritis (IBD w/ arthritis)

• Crohn’s or Ulcerative Colitis w/ peripheral arthritis or spondyloarthropathy

• Anterior uveitis will occur in 2-11% of individuals with IBD

  • insidious (no symptoms)

  • recurrent or chronic

  • bilateral

  • non-granulomatous

• Symptoms: none

• Presentation:

  • Absent circumlimbal hyperemia

  • Fine white keratic precipitates

  • Cells and flare in the anterior chamber

• Complications:

  • Secondary glaucoma

  • Cataract

• Treatment

  • Topical cycloplegic to prevent post. synechia

  • Topical corticosteroids

• Other ocular manifestations: Episcleritis, scleritis, keratitis

Behçet’s Disease

• Characterized by:

  • Oral and genital ulcers

  • Skin lesions

  • Ocular inflammation: anterior and posterior uveitis may be present

    • Anterior uveitis will be present in 19-31%

      • sudden onset

      • acute

      • non-granulomatous

• Symptoms: pain, photophobia, redness

• Presentation:

  • Circumlimbal injection

  • Cells and flare in the anterior chamber

  • Hypopyon may be present; can shift with head movement

• Complications:

  • Secondary glaucoma

    • from post synechia, peripheral anterior synechia, or neovascularization

      • neovascularization is more common with posterior segment involvement; ischemic retina

  • Iris atrophy

  • cataract

• Treatment:

  • Topical cycloplegic drop

  • Topical or systemic corticosteroids

Fuchs’ Heterochromic Iridocyclitis

• Typically unilateral but can be bilateral

• Affects males and females equally

• Onset: all ages

• Characterized by:

  • Iris heterochromia

    • Caused by iris atrophy of the anterior layers of the iris

    • Blue irides may appear darker and brown irides may appear lighter

  • Anterior uveitis

    • insidious

    • chronic

    • non-granulomatous

• Symptoms: none

• Presentation:

  • Small, diffuse, stellate keratic precipitates

  • Cells and flare

    • minimal flare

  • Iris nodules: Koeppe or Busacca nodules

    • tend to be small and transparent

  • Russell bodies: minute, crystalline, highly refractile deposits on the iris surface

• Complications:

  • Secondary glaucoma (59%)

  • cataract

• Treatment:

  • Short course of topical steroids when indicated

  • Treatment of the ocular complications

Sarcoidosis

• multisystem granulomatous disease that primarily affects the lungs, skin, eyes and lymph nodes

• Affects both males and females

  • Females are more likely to have ocular manifestations

• Affects all ethnicities but is most severe in African American individuals

• Onset occurs in two peaks: 20-30 year olds and 50-60 year olds

• Anterior uveitis is the most common ocular presentation

  • tends to be bilateral

  • onset is sudden or insidious

  • clinical course is acute or chronic

  • granulomatous

• Symptoms: asymptomatic (if insidious), pain, photophobia, redness

• Presentation:

  • Mutton fat keratic precipitates

  • Cells and flare

    • hypopyon uncommon

  • Iris nodules: Koeppe or Busacca

  • Tent shaped peripheral anterior synechia

• Work-up: Angiotensin converting enzyme (ACE), chest X-ray

• Complications:

  • Secondary glaucoma

  • Cataract

  • band keratopathy

• Treatment:

  • Topical cycloplegic drops

  • Topical corticosteroids

Vogt-Koyanagi-Harada Disease (VKH Disease)

• Autoimmune disorder that affects the eyes, auditory system, nervous system and skin

  • Melanin containing cells are commonly “attacked” in VKH

• More common in darkly pigmented individuals

• Affects females more than males

• Onset: second to fifth decade

• 4 Stages:

  • Prodromal stage: mimics a viral infection

    • Neurological and auditory manifestations are common

  • Acute uveitis stage: this stage may last for several weeks

    • bilateral

    • granulomatous

    • Posterior uveitis is commonly seen prior to anterior uveitis

    • mutton fat keratic precipitates and iris nodules

  • Convalescent: this stage will be seen one to three months after onset, may last for several months

    • Depigmentation of the skin and uvea will be seen

  • Chronic recurrent: this stage may interrupt convalescent

    • Anterior uveitis will be present in this stage (granulomatous + iris nodules)

    • Posterior segment involvement is rarely seen in here

• Complications: more common with long durations of the disease, multiple recurrences, and older age

  • cataract

  • Glaucoma

  • Retinal neovascularization

• Treatment:

  • Systemic: systemic corticosteroids

  • Ocular treatments: topical corticosteroids and topical cycloplegic drops

Uveitis from Herpes Simplex and Herpes Zoster

• causes 8% of uveitis cases

• The clinic course can be acute, chronic or recurrent

• Granulomatous

• Can cause anterior or posterior uveitis

• Presentation:

  • Decrease corneal sensation

  • Keratic precipitates will have a stellate appearance rather than an inferior deposition

  • Sectoral or non-sectoral iris atrophy

    • non-sectoral = HSV

    • sectoral = HZO

  • Acute uveitis will present with elevated intraocular pressure

    • d/t trabeculitis

• Complications

  • Secondary glaucoma

  • Hypotony

  • Iris atrophy

  • Cataract

• Treatment:

  • Oral antiviral medications

  • Topical corticosteroids