Uveitis

Introduction
Uveitis : inflammation of uveal tract (iris, ciliay
body & choroid)

It is classified anatomically to
1. Ant.uveitis (iritis)
inflammation of iris accompanied by increased vascular permeability which
allow both protein & WBCs to extravasate into the aqueous.
2 types : granulomatous (Sarcoidosis,Syphilis, Vogt-Koyanagi-Harada
disease Sympathetic ophthalmi Multiple sclerosis Lyme disease
Tuberculosis.) nongranulomatous.
circulating White cells could be seen in the aqueous humor of Ant.
Chamber using a slit lamp.
Protein which leaked from BV is picked out by its
light scattering properties appear as flare in the beam of slit lamp.
Anterior Uveitis with hypopyon in a patient with IBD
2. Intermediate uveitis
Inflammation of ciliary body (cyclitis),of the
pars plana (pars planitis) and of the vitreous
(vitritis)
3. Posterior uveitis
Inflammation of posterior uvea May also
involve the choroid(choroiditis) or
retina(retinitis)
Panuveitis :its when inflammatory
changes involve the anterior
chamber,vitreous,retina and/or choroid.
Epidemiology

Incidence 15/100000 (75% ant.uveitis)

8/100000 in US
50% of pts have ass. Systemic dis.
 Hx.

pt come to clinic complaining of:

o ocular pain (less frequent with post.U)
Abrupt onset dull aching eye pain worsen when touch the eye through
eye lid, may refered to temple or periorbital region
o Photophobia
o Blurring of vision Redness of the eye
HISTORY
o Respiratory symptoms (S.O.B , cough , nature of sputum)
..TB, Sarcoidosis.
o Skin problems acompained by uveitis
erythema nodosum (arms +chin) >>>> Sarcoid & Bechet
oral & genital ulcers >> Bechet
o Joint disease
AS.20% ant.uveitis
juvenule chronic arthrits
Reiter syndrome..40% ant.uveitis
o Bowel dis : IBD
 Signs
visual acuity may reduced
ciliary injection:mostly around limbus
Anterior uveitis:
Keratitic precipitates
inflam. Cells maybe visible as clumps on
endothelial of cornea (mostly Inferior)
On slit lamp exam.
flare & hypopyon in severe inflam.
Hypopyon highly suggestive for HLAB27 related & less
commonly with infectious iritis.
 Dilated vessels on iris
Posterior synechia.iris adhere to lens

Elevated IOP(inc aqeous protein or due to

occlusion of drainage angle by PAS)
 Intermediate and posterior uveitis
Cells in the vitreous

Retinal or choroidal foci of inflammation

Macular edema
 Grading of flare(flare refers to liberated protein from the inflamed
iris or ciliary body which gives the aqueous a particulate, or
smoky, appearance.)

0 Completely absent
1+ Barely present
2+ moderate
3+ marked
Gading4of
+ aqueous
Intense
cells (fibrin)

trace 1-5 cells

+1 . 6-15
+2 . 16-25
+3 . 26-50
+4 .. >50
Signs
 Signs

KP: fibrous deposits on the posterior

surface of the cornea, usually
associated with uveitis.

Both the size and

distribution of keratic
precipitates are helpful in
the differential diagnosis.
White, yellowish greasy precipitates of
inflammatory cells
Typically distributed in a wedge-shaped region
on the inferior corneal endothelium, known as
Arlt's triangle
Signs

annular s. adhesion of the

whole rim of the iris to the lens.
anterior s. adhesion of the iris
to the cornea.
peripheral anterior s.
adhesion of the peripheral iris to
the cornea.
posterior s. adhesion of the
iris to the capsule of the lens or
to the surface of the vitreous
body.
total s. adhesion of the whole
surface of the iris to the lens.
Signs
Signs
 Invetigations
Aim : determine systemic association
Investigation guided by past medical Hx &
review of system:
1. Pt with first episode of nongranulomatus iritis with unremarkable
past medical Hx & review of systemso investigations not
indicated
2. Recurrent persistant iritis with unusual severity, unresponsive to
medical therapy or bilateral.so here investigation is
needed
* At minmum CXR , RPR (rapid plasma reagin) test &
fluorescent treponemal antibody absorption (FTA-ABS FTA-ABS)
should be ordered
 ACE test , lysozyme level & ESR for
evaluating sarcoidosis
HLA B27 typing

RF & ANA if we suspect juvenile idiopathic

arthritis
 Imaging studies
1. CXR to rule out sarcoidosis & TB
2. chest CT done if we have ve CXR but
sarcoidosis still highly suspected
3. Sacroiliac radiograph , if AS is suspected
Procedures

if patient have secluded pupil from extensive

post. Synechia , iris bombe with closed angle
glucoma ..here iridotomy maybe needed
Etiology
Individual forms of uveitis may be
distinguished on the basis of location within
the eye, onset, symmetry and continuity of
inflammation, associated complications, and
distribution of cells along the corneal
endothelium.
Etiology
Infections

Systemic immune-mediated disease (40%)

Syndromes confined primarily to the eye

Masquerade syndromes

30% of patients dont fit an well-defined

disease category .
Infections
Cytomegalovirus (CMV) in adults is found almost exclusively in the
immunocompromised host, especially patients with HIV infection who
have extremely low CD4 counts

Toxoplasmosis is a surprisingly common cause of uveitis in the normal

host. In many instances, it is presumed to be a reactivation of a
congenitally acquired infection. It is suspected on the basis of a typical
chorioretinal lesion; the diagnosis is supported by serology. Most
chorioretinal scarring from toxoplasmosis is due to infection during
gestation, but scarring is increasingly recognized as a result of recent
infection . Patients may complain of hazy vision and floaters.retina is the
target choroiditis is secondary.

Syphilis accounts for less than 1 percent of patients with uveitis in most
large series. It may present in a variety of forms, including posterior
uveitis, such as a chorioretinitis or retinal vasculitis
CMV retinitis(cottage cheese
lesion)
Toxoplasmosis
Infections
Tuberculosis is an uncommon cause of uveitis in North American
referral centers. It should be considered in the differential diagnosis
when the uveitis worsens despite glucocorticoid therapy. Additional
factors that raise suspicion for this diagnosis are active tuberculosis
elsewhere in the body, cachexia, homelessness, a granulomatous
appearance for the ocular inflammation, or immunosuppression. In
some geographic areas such as Saudi Arabia, tuberculosis is
considered a common cause of uveitis.

Both herpes simplex and herpes zoster can cause a keratouveitis, an

inflammation of the cornea along with uveitis that is primarily anterior.
The presence of cutaneous vesicles, characteristic corneal changes,
reduced corneal sensation, elevated intraocular pressure, and iris
atrophy may be clues to the diagnosis. Both herpes simplex and zoster
can also cause a retinitis known as acute retinal necrosis. This is a rare
but treatable cause of visual loss.
Iris atrophy in a patient with herpes simplex virusassociated
.anterior uveitis
Immune-mediated systemic
disorders
Spondyloarthritides (SpA), such as ankylosing spondylitis and
reactive arthritis, are the most common systemic immune
disorders associated with uveitis in North America and Europe

20-40 %

Male >female

typically unilateral, and tends to resolve within three months of

its onset. Recurrences are common, and can occur in the
contralateral eye.

The prognosis for this form of uveitis is generally excellent

provided that acute attacks are treated early and vigorousely
AS

Fibrin clot and posterior synechiae in a patient with

acute, anterior uveitis and ankylosing spondylitis.
Immune-mediated systemic
disorders
7% psoriatic arthritis and 2 to 9 % of patients with IBD may develop uveitis.

uveitis associated with IBD or psoriatic arthritis is frequently bilateral, posterior to

the lens, insidious in onset, chronic in duration, and more common in females
than males .
In 10 of 17 patients in one series, the uveitis preceded signs of IBD

Sarcoidosis accounts for a significant percentage of patients with uveitis in most

series from referral centers .
Approximately 20 % develop eye disease as their initial presentation of sarcoid.
The eye disease can take on many forms, including uveitis, dry eyes, optic
neuritis, lid inflammation, or orbital disease .
Uveitis in patients with sarcoid is frequently associated with retinal vasculitis,
which may be either perivascular or involve retinal vascular changes The eye
disease may persist despite the resolution of adenopathy [ (
Immune-mediated systemic
disorders
as many as 80 percent of patients with Behcet's disease develop uveitis .
Uveitis is often the dominant manifestation of this disease, and is typically bilateral. Similar
to HLA-B27-associated iritis, it is frequently episodic, but unlike HLA-B27-associated
disease, the uveitis generally does not resolve completely between episodes. Retinal
vasculitis is a frequent manifestation of Behcet's disease .
The uveitis typically leads to blindness if the eye inflammation is not treated

Juvenile idiopathic arthritis (JIA) may be associated with uveitis, particularly in the subset
of patients with pauciarticular disease and a positive antinuclear antibody. The onset of
uveitis is more common in younger children and, in the majority of patients, can be
asymptomatic. (white eye but wuth signs of uveitis present )
The uveitis associated with JIA is usually bilateral, insidious in onset, chronic in duration,
and anterior. The eye disease is commonly associated with complications such as band
keratopathy, posterior synechiae , cataract formation, and glaucoma. The uveitis
sometimes lasts for decades, long after the joint disease has disappeared.
Behcet

Male,young,bilateral , hypopyon
Immune-mediated systemic
disorders

(SLE) can involve the eye in a variety of forms

.Dryness is the most common ocular manifestation;
cotton wool spots) occur in about 7 percent of
patients and indicate local retinal ischemia .
Anterior uveitis is a rare manifestation.'.)
Uveitis is infrequently associated with other
systemic vasculitides, including polyarteritis and
granulomatosis with polyangiitis (Wegeners). More
typical vision-threatening manifestations of
granulomatosis with polyangiitis (Wegeners) are
scleritis and orbital disease
Vogt-Koyanagi-Harada
is the second leading cause of uveitis in Japan after Behets
syndrome
Multisystem disorder
Bilateral panuveitis with exudative retinal detachment followed by
neurological and cutananeous manifestations (baldness and loss
of lash and skin pigment)
F>M
20-50s
Prodromal stage(fever,headache,tinnitus and CSF
pleocytosis),weeks later bilateral posterior uveitis and
subsequebt datachment.few weeks later the daetachment
subsides with pigmentary choriodal changes and depigmentation
of the retina.
VKH skin changes (vitiligo of
the lashes and eyebrows)
Fuchs heterochromic uveitis
Rare , chronic ,young adults
Unknown cause , no systemic association

Mild ant uveitis,no signs of conjunctival inflamation, no ant synechia

KPs diffusely distributed over the cornea

Heterochromic iris due to loss of pigment epithelial cells.

Inflamed vitreous

70% catarct

Steroids arent effective and not prescribed,catarct surgery is done when

indicated,and patients usually respond well
Sympathetic ophthalmitis
rare, bilateral granulomatous uveitis
after either surgical or accidental trauma to one eye.
The ocular inflammation in the fellow eye becomes apparent usually within 3 months
after injury.
Clinical presentation is an insidious or acute anterior uveitis with mutton-fat keratic
precipitates.
The posterior segment manifests moderate to severe vitritis, usually accompanied by
multiple yellowish-white choroidal lesions. Evidence suggests that it represents an
autoimmune inflammatory response against choroidal melanocytes mediated by T
cells.
Diagnosis is based on clinical findings and a history of previous ocular trauma or surgery.
Other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease,
sarcoidosis, tuberculosis, and syphilis should be considered.
Treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents
with high dose oral corticosteroid as the drug of choice.
However, if the inflammation cannot be controlled, cyclosporine is then used. Other
immunosuppressive agents, such as chlorambucil, cyclophosphamide or azathioprine,
may be necessary for the control of inflammation.
The role of enucleation after the diagnosis of sympathetic ophthalmia remains
controversial. Visual prognosis is reasonably good with prompt wound repair and
appropriate immunomodulatory therapy.
 Treatment
Aims:
1. relief pain & inflammation
2. prevent ocular structural damage
3. prevent visual loss & retinal or optic
damage
Medication:

cycloplegia
Long acting cycloplegic agents (cyclopentolate ,
hematropine )used to relief pain& photophobia by
medriasis .
 Corticosteroids

1. Topical , is the mainstays of therapy used

aggressively esp. in initial phases of
therapy ,
if no response in 7-10 days we use :
2. subconjunctival injection (celestone) , used
if pt poorly complies to topical type or iritis not
respond to topical ttt alone
3. oral corticosteroid maybe necessary in
severe cases of iritis and post uveitis
 Topical aqueous suppressant
in case of elevated IOP

Little or No benefit of using NSAIDS in iritis

Ant.uveitis:
dilating pupil to prevent synechia
(homatropine , cyclopentolate or atropine
drops)
To break synechia

1. initially .intensive cyclopentolate ,

phenylphrene or tropicamide drops
2. if resistant synechia . Subconjuctival
mydriatics
 post.Uveitis:

visual loss occur due to destructive process

by retinitis or macular edema due to fluid
acumulation. So here we use
antiviral, antibiotics or systemic steroids
some rare severe uveitis may need other
immunesuppresive agents (Azathioprine,
cyclophosporin)
 Follow up:
* reexamine pt 2-3 wks later to ensure no
residual inflammation present
Complication

* recurrent episodes of iritis & the subsequent

therapy may lead to cataract or glucoma
Prognosis

* Most pt expect to have recurrent iritis.

* Visual prognosis is good in absence of either
cataract or glucoma.
 Specific conditions ass. with uveitis
Disease Hx Signs Investi Treatment Prognosis
gati-
ons
AS Ant.uveitis Typical signs of HLAB2 Ocular ttt Recuurent
Stifness at Ant.uveitis 7 Anti- attacks
rest Sacroili inflammator But good for
Backache -ac physiotherapy vision
X-ray
Rieters Male , 40% with
disease HLAB27 Acute ant uveitis
Urethritis ,
arthritis,
conjunctivitis

Juvenile Chronic White eye Ocular ttt

chronic asymptomati 70% shows ANA Systemic ttt
arthritis c ant. Uveitis bilateral of arthritis
Profound
visual defect

Fuchs Blurred vision Mild ant. uveitis Not respond

Heterochromi & floaters Heterochromic iris to steroid
c uveitis (not present Catract 70% Respond to
with typical Glucoma,KPs, cataract
iritis Hx.) No post. Synechia surgery
 Dis. Hx Signs investigation Treatment
s
Toxoplasmosis Mostly Creamy foci +ve Systemic
Congenital(50- of toxoplasmosis steroid
75%) Inflammatory Antibody test &
Hazy vision , Cells at the is suggestive clindamycin
floaters , red & margin
painful eye At the margin
of
chorioretinal
scar