CHRONIC UVEITIS

Presented by – Dr Sushma Rose

Tirkey M.S.(Opthal)
 UVEITIS ASSOCIATED WITH NON-INFECTIOUS
SYSETEMIC DISEASES
SARCOIDOSIS
• Sarcoidosis is a common,
idiopathic, multisystem
disorder characterized by
the presence of non-
caseating granulomata in
the lungs and other organs.
• Presentation : Presentation
is usually in one of the
following two ways:
• Acute onset : During the
third decade characterized
by erythema nodosum,
parotid enlargement and
hilar lymphadenopathy.
• Insidious onset : During the fifth decade characterized
by fatigue, dyspnoea and arthralgia.
Diagnostic Tests
• Chest Radiographs will be abnormal in over 90% of
patients. Shows bilateral hilar lymphadenopathy
• Biopsy of the conjunctival nodules, enlarge lymph
node, lung , skin lesions
• The Kveim-Siltzbach test is positive in 85-90%
• Serum Angiotensin-converting enzyme (ACE) is
usually elevated in patients with active sarcoidosis
• Calcium assays show abnormal, Hyper – calciuria is
common
 OCULAR FEATURES
• The eye is involved in about
30% of patients with systemic
sarcoidosis. Ocular
inflammation is usually
unilateral and, as the disease
becomes chronic, bilateral
involvement usually develops.
•  Eyelid Lesions
• CONJUCTIVA L GRANULOMA
Consist of violaceous sarcoid
plaques (lupus pernio), sarcoid
granulomata of the lid
margins.
• Anterior Segment Lesions
• Involve the conjuncitva,
episclera and, rarely, the
sclera. Infiltration of the
lacrimal gland may result in
keratoconjunctivitis sicca. INFILTRATION OF THE LACRIMAL
GLAND
 Anterior Uveitis
• Acute or Chronic
• Acute iridocyclitis : It is
unilateral, associated with
hilar lymphadenopathy and
erythema nodosum
• Chronic iridocyclitis : It is
more common and bilateral
and presents with typical
features of granulomatous
iridocyclitis associated with
chronic lung fibrosis. The
intraocular inflammation may
be difficult to control and
complications such as band
keratopathy, complicated
cataract and secondary
glaucoma are frequent.
 COMPLICATIONS

Complicated Cataract Band Keratopathy

Uveoparotid fever
(Heerfordt’s syndrome)
Bilateral granulomatous
panuveitis, painful
enlargement of parotid
glands. Cranial nerve palsies,
Secondary Glaucoma skin rashes, fever and malaise.
 Posterior Segment
• Segment is involved in
about 25% of patients
and is associated with
anterior uveitis.
• Vitreous Changes :
• Diffuse vitritis : Is the
most common and is
usually most marked
inferiorly. Vitritis and Snowballs
• Snowball opacities may
be present occasionally
 Fundus Changes
• Periphlebitis is
characterized by
‘candlewax drip-pings’.
• Complications of
Periphebitis Candlewax
periphebitis are retinal dripping
branch vein occlusion,
peripheral
neovascularization and
disc neovascularization.

Neovasularization
• Retinal granulomata :
Sarcoid nodules may be
located on the retinal
surface and they may also
extend into the vitreous.
Preretinal nodules are
typically discrete, grey-
white, and located inferior
and anterior to the equator
(Lander’s sign).
• Acute sarcoid retinopathy –
Vitreous haze, candlewax Preetinal nodules in Sarcoidosis
drippings, retinal and (Lander’s Sign)
preretinal granulomata, and
retinal haemorrhages.
• Small choroidal granulomata
– Common
• Large choroidal
granulomata – Rare
• Optic nerve lesions
(a)Focal granulomata
may involve the
optic nerve. Large choroidal Granuloma

(b) Papilloedema
(c) Neovascularization
of the optic nerve
head

Optic Disc Granuloma

 Treatment
• Topical, periocular and systemic steroids constitute
the treatment of sarcoid uveitis, depending upon the
severity.
• Sytemic steroids may be necessary in patients with
severe posterior segment disease, particularly if the
optic nerve is involved.
 BEHCETS DISEASE
• Behcets disease is an
idiopathic multisystem
disorder which typically
affects young men.

Presentation
• Third and fourth decades
with recurrent oral
ophthous ulceration
associated with :
• Recurrent genital
ulceration
• Erythema multiforme
• Eye involvement
 Etiology
• Associated with an increased
prevalence of HLA – B5.
• Basic lesion is an obliterative
vasculitis caused by abnormal
circulating immune complexs
Ocular Features
• 70% patient with Behcets
disease develop recurrent,
bilateral non-graulomatous,
intraocular inflammation.

• Acute recurrent iridocyclitis :

• Transient hypopyon is
common.
• It may also be associated
with posterior uveitis,
vitritis, periphlebitis retinae
and retinitis in the form of
white necrotic infiltrate.

Treatment of Posterior Uveitis

• Sytemic Steroids : To
control posterior segment
inflammation
• Chlorambucil : Effective in
about 75% of cases
• Cyclosporin : Potent
affecting both the cellular
and humoral arms of the
immune response.
• Plasma exchange
 Vogt – Koyanagi – Harada
Syndrome 

• The vogt – koyanagi – syndrome

(V-K-H) is an idiopatic,
multisystem disorders which
typically affect Japanese people
who are usually positive for HLA –
DR4 and DW15
 
CLINICAL FEATURES

• Skin and hair changes –

• Alopecia – 60% of patient
• Poliosis – Common, develops
several weeks after the onset of
the disease.
• Vitiligo – (Patches of skin
depigmentation usually follows
the onset of visual symptoms by
several weeks.
 
 NEUROLOGICAL FEATURES
• Irritation – Headache and
stiffness
• Encephalopathy –
Convulsions, cranial nerve
palsies and paresis.
• Audiotory symptoms –
Tinnitus, vertigo and
deafness
• Cereboreospinal fluid
lymphocytosis

Ocular Features
• Chronic anterior
granulomatous
iridocyclitis
 Posterior segment
involvement
• Multifocal choroiditis
associated with disc
hyperemia or oedema.
• Development of multi focal
detachment of the sensory
retina.
• V-K-H SYNDROME
Subdivided into
(A) Vogt – Koyanagi syndrome
- Skin changes and anterior
uveitis
(B) Harada’s disease –
Neurological features and
exudative retinal
detachment
Predominate
 UVEITIS ASSOCIATED WITH
ARTHRITIS
ANKYLOSING SPONDYLITIS
• Common, Idiopathic, chronic
inflammatory arthritis which
primarily involves the axial
skeleton
• More common in males  
Presentation Fixed Flexion Deformitiy
• Second and third decades.
• Gradual onset of chronic
bachache and stiffness  

OCCULAR FEATURE
• Acute, recurrent, non
granulomatous iritis – Occurs
in 30%
• Mostly affects one eye at a
time Bilateral Sclerosis & Erosion of
Sacroiliac Joints & Bony Fusion of Spine
 TREATMENT

• Same as usual treatment of anterior

uveitis
• Long term aspirin and indomethacin may
decrease the recurrences
REITER’S SYNDROME  

• Reiter’s syndrome
consist of a triad of –
• Uveitis
Acute Arthritis of Ankles
• Conjunctivitis
• Arthritis  
• Disease is uncommon
and affective men
more. About 75%
patient are positive
for HLA – B27 Conjunctivitis
 Presentation : Third decades
• Acute arthritis – Affecting knees and ankles
• Acute mucpurulent conjunctivitis – Bilateral
associated with epithelial keratitis with
subepithelial opacities
• Acute non-granulmatous – Iridocyclitis occurs
in 20% of the patients.
Treatment
 
Oral tetracycline 250 mg qid for 10 days.
Calcaneal Spur Painless Mouth Ulcer

Circinate Balanitis Keratoderma Blenorrhagica

 JUVENILE CHRONIC
ARTHRITIS (JCA)
• Uncommon idiopatic,
inflammatory arthritis at
least 3 months duration
developing in children
before the age of 16
years.
• Involves multiple joints
knees, elboo, ankle,
interphalangeal joints
• The female and male
ratio is 3:2
• JCA reffered to as
Juvenile rheumatoid
arthritis
 The 3 types of presentation

1. Systemic onset JCA – Occurs

about 25%, high remittent
fever
• Transient maculopapular rash
• Generalized lymphadenopathy
• Heapatosplenomegaly
• Polyarthritis : The condition is
labeled as “Still’s disease”

2. Polyarticular onset of JCA -

Occurs 20% in patient
• Artheritis involves commonly
the knees followed by the
wrists and ankles
• Systemic features and mild or
absent
• Uveitis is uncommon
3. Pauciarticular onset
of JCA
• Occurs in about 60% in
cases.
• Arthritis most commonly
involves knees
occassionaly only a single
finger and
• toes is affected
• Systemic features are
absent
• Uveitis is common
• Early onset disease –
Positive for antinuclear
antibodies (ANA), HLA –
DW5 and HLA – DPW2
 OCULAR FEATURES
• Anterior Uveitis is chronic
non – granulomatous and
bilateral in 70% of cases
• Onset of uveitis is
asymptomatic and the
eye is white even in the
presence of severe
uveitis.
• Therefore, SLE is
mandatory in children
suffering from JCA
• Complications : Posterior
Synechiae
• Complicated cataract
• Band –shaped
keratopathy are common
 TREATMENT

• Topical steroids is usually effective in the

most patients.
 UVEITIS IN PARASITIC
INFECTIONS
• Toxoplasmosis
• Toxoplasma gondii is an
obligate intracellular
protozoan.
• The cat is the definitive
host and other animals
such as mice cattle,
sheep, pigs and humans
are intermediate hosts.
• The disease primarily affects CNS
and retina. Human may become
infected by ingestion under cooked
meat and sporocytes.
• Systemic toxoplasmosis occurs in
humans in two forms :
• Congenital
• Aquired
• Congenital Toxoplasmosis :
Toxoplasmosis is transmitted to the
fetus through the placenta when a Hydrochephalous
pregnant woman contracts the acute
form.
• Tried of congenital toxoplasmosis –
Convulsions, Chrorioretinitis and
Intracranial calcification.
• In active stage – The typical lesion is
necrotic granulomatous
retinochoroiditis involving the
macular region.
• Inactive stage – Bileteral healed
punched out heavily pigmented
chorioretinal scar in the macular
area.
Macular Scar
• Aquired Toxoplasmosis: Chorioretinal lesion similar
to congenital toxoplasmosis.
• Recurrent Toxoplasmic retinochoroiditis – The
recurrences usually take place between the ages of
10 to 35 years (average age 25 years) when the
cysts rupture and release hundreds of parasites into
the normal retinal cells.
• Primary lesion is an inner retinitis and inflammatory
reaction seen is in choroid, iris and retinal blood
vessels is belived to be immune origin and not the
result of direct infestation.
 CLINICAL FEATURES
• Iridocyclitis – Common and
may be granulomatous or
non-granulomatous.

POSTERIOR UVEITIS
• Whitish yellow, slightly
Unifocal Retinitis Adjacent to
raised area of infiltration Old Scar
located near the margin of
old punched out scarred
lesion in the macular region
associated with severe
vitritis. There may be
associated non-
granulomatous type of mild
anterior uveitis.
Very Severe Vitritis & Head
Light in Fog
 Diagnosistic Tests
• Indirect immunofluorescent antibody tests
• Haemagglutination tests
• Enzyme – linked immunosorbent assays
Treatement
• Topical and systemic steroids.
• Antitoxoplasmic drugs
• Spiramycin
• Clindamycin
• Sulfadiazine
• Pyremethamine
• Clindamycin – 300 mg four times daily is given orally
for 3-weeks.
• Sulfadiazine – Oral dose 2mg followed by 1g four
times daily for 3-4 weeks.
• Pyremethamine – The loading dose is 75-150 mg
followed by 25-50 mg daily for 3-4 weeks.
• Co-trimoxazole – Is the combination of trimethoprim
160 mg and sulphamethoxazole 800mg twice daily for
4-6 weeks.  
• Adjunctive therapy :
• Laser photocoagulation
• Cryotherapy
• Vitrectomy
RARE IDIOPATHIC SPECIFIC
UVEITIS SYNDROMES
FUCHS’ UVEITIS SYNDROME (FUS) or
Fuchs’ heterochromic cyclitis is a
chronic, non-granulomatous,
anterior uveitis which has a
insidious onset .
• Typically ouccurs unilaterally in
middle-aged persons.
• Disease is characterised by
a. Heterochromia of iris
b. Diffuse stromal iris atrophy
c. Fine KPs at back of cornea
d. Faint aqueous flare
e. Absence of posterior synechiae
f. Rubeosis iridis
g. Some times associated with
neovascularisation of the angle
Early Development of Complicated Cataract &
Secondary Glaucoma
Usually open angle type

Treatment
• Topical steroids
• The patient should be examined at approximately 6 monthly
intervals to detect glaucoma.
SYMPATHETIC OPTHALMITIS
It is a serious bilateral
granulomatous panuveitis
which follows a penetrating
ocular trauma and surgery.
The injured eyes is called
‘exciting eye’ and the fellow
eye which also develops
uveitis is called
‘sympathizing eye’.
• 65% of cases of sympathetic
uveitis – between 2 weeks
and 3 months after injury.
• 90% of cases occur within
the first year.
 Pathology
Dalen-fuchs’ nodules
are formed due to
proliferation of the
pigment epithelium (of
the iris ciliary body and
choroid) associated
with invasion by the
lymphocytes and
epitheloid cells. Retina
shows perivascular
Choroidal thickening by diffuse
cellular infiltration granulomatous infiltration. Dalen
known as peri –Fuchs nodules formed between
retinal pigment epithelium and
sypathetic Bruch’s membrane
perivasculitis.
 Predisposing Factors
1. It almost always follows a penetrating wound.
2. Wounds in the ciliary region.
3. Wounds with incarceration of the iris. Ciliary
body or lens capusle.
4. It is more common in children than in adults.
5. It does not occur when actual suppuration
develops in the injured eye.
 Clinical Feature
1. Exciting eye – It shoizws
low grade plastic uveitis
which include ciliary
congestion lacrimation
tenderness, KPs may be
present at the back of
cornea (dangerous sign)
2. Sympathizing (sound) eye:
It is usually involved after 4-
8 weeks of injury in the
other eye. Clinical picture of
the iridocyclitis in
sympathizing eye can be
divided into two stages.
1. Prodermal Stage – Symptoms :
• Photophobia
• Transient Indistinctness of near objects due to
weakness of accommodation are the earliest
symptoms
Sign:
• Mild ciliary congestion
• Slight tenderness of the globe
• Few KPs at the back of cornea.
• Fine vitreous haze and disc oedema is seen
occasionally.
2. Fully Developed Stage:
Sign and symptoms are
same as acute plastic
iridocyclitis.

Posterior Segment:
Small, deep, yellow-white
spots corresponding to
Dalen-Fuchs’ nodules,
scattered throughout both
fundi.
 TREATMENT
1.Enucleation – Within 2 weeks of the injury
will prevent sympathetic uveitis in most
cases.
2.Steroid therapy by all possible routes of
administration. Once the uveitis is control
treatment can be gradually tapered but any
acute exacerbations should be treated
intensively.
3.Immunosuppressive therapy with
chorambucil, cyclophosphamide or
cyclosporin may be required in severe
steroid – resistant cases.