Transcript Basic Uveitis Lect

Uvea & Sclera
SINA A. SHOOL MD
www.drsinashool.blogspot.com
Uveitis – “inflammation of
the uveal tract”
1) Anatomic classification
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Anterior Uveitis: iris & Anterior part of
the ciliary body (pars plicata
Intermediate Uveitis: posterior part of
the ciliary body (pars plana)
Posterior Uveitis
Panuveitis
Anatomical - Anterior
Uveitis
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Juvenile Rheumatoid Arthritis
Fuchs’ heterochromic Iridocyclitis
Ankylosing Spondylitis
Reiter’s Syndrome
Anatomical Intermediate Uveitis
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Pars Planitis
Sarcoidosis
Multiple Sclerosis
Lyme Disease
Anatomical - Posterior
Uveitis
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Toxoplasmosis
Histoplasmosis
Toxocariasis
Serpiginous chorioretinitis
Birdshot retinochoroiopathy
Anatomical - Diffuse
Uveitis
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Sympathetic Ophthalmia
VKH syndrome
Behcet’s disease
(2) Clinical Classification
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Acute Uveitis: sudden, symptomatic
onset; persists for <6 weeks
Chronic Uveitis: insidious onset, may
be asymptomatic; persists for months
(3) Etiological Classification
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Exogenous Uveitis: due to external
injury to the uvea or invasion of microorganisms from the outside
Endogenous Uveitis: caused by microorganisms or other agents from inside
the patient
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(4) Pathological Classification
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Granulomatous Uveitis
- epithelioid and giant cells,
lymphocytes
Non-granulomatous Uveitis
- lymphocytes and plasma cells
(5) Pathophysiologic
Mechanisms (Opremcak)
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Traumatic
– Surgical vs. non-surgical
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Infectious
– Bacterial, Viral, Fungal, Protozoal,
Helminthic, Insect, Animal
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Immunologic
Masquerade Diseases
Immunologic
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Type I hypersensitivity disorders
– Ig E- mediated
– Mostly ocular surface disease hay fever,
vernal & atopic keratoconjunctivitis
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Type II
– Antibody dependent cellular cytotoxicity
(autoantibodies)
– Ocular Cicatricial Pemphigoid; Lensinduced Uveitis
Immunologic
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Type III
– Immune Complex formation & deposition
– Ankylosing Spondylitis, SLE, JRA, Behcet’s
disease, PAN, Wegener’s granulomatosis,
inflammatory bowel disease, other collagen
vascular diseases
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Type IV
– Cell mediated immune response
– Interleukins
– Contact dermatitis, Phylectnulosis, , Fuchs HI,
Sympathetic Ophthalmia, VKH, Birdshot
retinochoroidopathy, Pars Planitis, Leber’s
Idiopathic Stellate Neuroretinitis, Sarcoidosis
Antigen
HLA-A1
HLA-A11
HLA-A29
HLA-B51
HLA-B7, DR2
HLA-B8
HLA-B12
Disease
Relative Risk
Sarcoidosis
Sympathetic
Ophthalmia
Birdshot
50-224
Retinochoroido
pathy (>97%)
Behcet’s Disease
4-6
POHS
12
Anterior Uveitis
5
Behcet’s
3-4
Disease(oral)
OCP
Antigen
HLA-B27
HLA-B53
Disease
Relative Risk
Ankylosing
100
Spondylitis (90%)
Reiter’s syndrome
40
(76%)
Acute Anterior
10
Uveitis (55%)
Inflammatory
Bowel Disease
Psoriatic Arthritis
Bechet’s Disease
VKH,
75
glaucomatocylitic
75
uveitis
Masquerade Syndrome
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Vascular tumors
Infectious
Congenital anomalies
Metabolic/Degenerations (amyloidosis,
retinitis pigmentosa)
Neoplastic Disease (lymphoma,
leukemia)
Clinical Features
Anterior Uveitis
Symptoms Photophobia, pain, redness,
decreased vision (Acute)
*Minimal symptoms in chronic type
Signs
Circumcorneal injection
Keratic precipitates (cellular
deposits on the corneal
endothelium)
Iris nodules: Koeppe & Busacca
Aqueous cells
Aqueous flare: leakage of proteins
Posterior synechiae
Grading System for Anterior
Chamber Flare & Cell
Aqueous Flare
0
Optically empty
1+
Faint
2+
Moderate- clear iris
& lens
3+
Marked- hazy iris &
lens
4+
Intense- fibrin with
no motion of cells
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Aqueous cell (1x3 mm
beam @ 5-10 °)
0
0
Rare
Rare
Occasional 1-2 cells
Trace
2-4 cells
1+
5-15
2+
16-25
3+
26-60
4+
> 60 cells
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Limbal Hyperemia=
Intraocular Inflammation
Clinical Features
Intermediate Uveitis
Symptom Floaters
s
Decreased vision (if with CME)
Signs
Vitritis: cellular infiltration of the
vitreous
+Vasculitis
+ few cells in the anterior
chamber
Grading System for Vitreous
Cell and Opacification
Flare (Vitreous
Opacification)
0
Good View of NFL
1+
Clear ON & vessels
but hazy NFL
2+
ON & vessels hazy
3+
ON only
4+
No ON
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Cell (1x3 mm beam in
anterior vitreous)
Trace
0-10 cells
1+
10-20
2+
20-30
3+
30-100
4+
> 100 cells
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Clinical Features
Posterior Uveitis
Symptoms
Floaters and decreased vision
Signs
Cells, flare, opacities in the
vitreous
Choroiditis: yellow or greyish
patches
Retinitis: whitish, cloudy retina
Vasculitis
Phlebitis (veins); Arteritis
(arterioles)
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Keratouveitis: with corneal
involvement
Sclerouveitis: with scleral involvement
Keratosclerouveitis
Anterior Uveitis
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Juvenile Rheumatoid Arthritis
Fuchs’ heterochromic Iridocyclitis
Ankylosing Spondylitis
Reiter’s Syndrome
Anterior Uveitis
1) Uveitis Associated with Joint Disease
a) Juvenile Rheumatoid Arthritis (JRA)
- chronic, bilateral, non-granulomatous
- arthritis of at least 3 mos’ duration in a
child less than 16 years
- females
- insidious onset
- uveitis may precede the onset of arthritis
JRA (cont)
- types of arthritis:
pauciarticular onset <4 joints
polyarticular onset
systemic onset
> 5 joints
Anterior Uveitis
2) Uveitis Associated with Joint
Disease(cont)
b) HLA-B27-associated
- Ankylosing spondylitis
- Reiter’s syndrome
- Psoriatic arthritis
- Inflammatory Bowel Disease (IBD)
HLA-B27-associated
Ankylosing Spondylitis
inflammatory arthritis
affecting axial
skeleton
Fixed flexion
deformity due to
fusion of spine and
sclerosis of sacroiliac
joints
Klebsiella &
Chlamydial infections
HLA-B27-associated
Reiter’s Disease
- Triad: urethritis, conjunctivitis, arthritis
- Extraarticular features:
Mouth ulcers, skin lesions (keratoderma
blenorrhagica), penile lesions
(circinate balanitis), nail changes,
cardiovascular changes, genitourinary
lesions
HLA-B27-associated
Psoriatic Arthritis
- Psoriasis: skin
disease due to
epidermal
hyperproliferation
HLA-B27-associated
Inflammatory bowel disease
- Crohn’s disease and ulcerative colitis
- Gastrointestinal and articular
manifestations
- Systemic manifestations include skin
lesions, oral ulcerations, hepatobiliary
disorders, urogenital involvement
Anterior Uveitis
3) Fuch’s Heterochromic
Iridocyclitis
- unilateral, insidious,
third or fourth decade
- No symptoms; low
grade uveitis
- Early cataract
- Fine, stellate, evenly
distributed deposits on
the posterior corneal
surface; iris atrophy;
telangiectatic vessels in
the angle
- Synechiae is rare
Fuch’s Heterochromic
Iridocyclitis
Anterior Uveitis
4) Herpetic Uveitis
- unilateral, high IOP, decreased corneal
sensation, iris atrophy
- Herpes simplex, Herpes zoster
- Treatment: oral anti-herpetic (acyclovir),
topical steroids, + anti-glaucoma
*posterior pole involvement: acute retinal
necrosis (ARN)
Posterior Uveitis
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1)
2)
3)
4)
usually associated with systemic
disease
Clues that can guide:
Morphology of the lesion
Age of the patient
Unilateral/Bilateral
Course of the disease
Posterior Uveitis
1)
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Ocular Toxoplasmosis
Toxoplasma gondii, obligate intracellular
protozoan
Most common cause of posterior uveitis in
U.S.
Domestic cat definitive hosts
Treatment: pyrimethamine, sulfadiazine,
clindamycin,
trimethoprim/sulfamethoxazole + steroids
Posterior Uveitis
2) Ocular Toxocariasis
- Toxocara canis,
intestinal parasite
of dogs
- Young children
- Infection by pica or
close contact with
puppies
- Usually unilateral
- Inflammation is
caused by death of
the larvae in the
retina
3) CMV retinitis
- opportunistic infection
usually found in AIDS
patients
- Retinal necrosis
- IV ganciclovir or
foscarnet; intravitreal
ganciclovir
4) Adamantiades-Behcet’s Disease
- idiopathic multisystem disorder
- Pathology: widespread vasculitis
- Triad: recurrent ocular inflammation,
skin lesions, recurrent oral & genital
ulcers
Panuveitis
1) Sympathetic
Ophthalmia
rare,
granulomatous,
bilateral
Follows a
perforating eye
injury, or surgery
Probably due to
hypersensitivity to
pigment-bearing
cells in the uvea
Sympathetic Ophthalmia
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Injured (exciting eye)
becomes inflammed
followed by fellow
(sympathizing) eye
Early enucleation of
exciting eye (within 10
days from injury)
Advanced cases:
systemic steroids or
chemotherapy
Histopathology
Dalen Fuchs’ Nodule
Granulomatous
Inflammation of Choroid
Panuveitis
2) Tuberculous Uveitis
- can occur even
without pulmonary
involvement
- Nodules; no specific
findings
- Tx: antituberculosis
drugs
Panuveitis
3) Vogt-Koyanagi-Harada
- systemic disorder
involving multiple
organ systems:
- Ocular (panuveitis,
exudative RD, optic
disc hyperemia)
- Auditory (tinnitus,
deafness)
- Nervous (headache,
meningismus, CSF
lymphocytosis)
- Integument (alopecia,
poliosis, vitiligo)
VKH
-
autoimmunity to
melanocytes in the
uveal tract and
integumentary
system possible
Areas of choroid retinal
adhesions with focal
proliferation of the retinal
pigment epithelium.
Lymphocytic infiltrates and
edema are noted
throughout the detached
choroid
Poorly defined nests of
epithelioid cells present
throughout the thickened
choroid with a moderate
lymphocytic infiltrate
4) Syphilis
- great imitator
- No pathognomonic
signs
Complications of Uveitis
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Band Keratopathy
calcium deposition in anterior layer of
the cornea (Bowman’s layer)
Treatment: ethylenediaminetetraacetic
acid (EDTA)
Complications of Uveitis
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Synechiae
-Peripheral anterior synechiae 
impede aqueous outflow: glaucoma
-Posterior synechiae
Seclusio pupillae: 360 posterior
synechiae  forward bulging of the
iris due to fluid accumulation (iris
bombe) glaucoma
Complications of Uveitis
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Cataract
-Interference with lens metabolism or
steroids
-Eye should be quiet for at least 3
months before undergoing cataract
extraction
-not all patients can tolerate IOL
implantation
Complications of Uveitis
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Retinal scars
Central vision may be spared if the
macula has not been involved
Peripheral scars may cause scotomas
Complications of Uveitis
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Retinal Detachment
Tractional: Result of traction on the
retina by vitreous strands
Serous: Fluid accumulation under the
retina
Complications of Uveitis
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Cystoid Macular Edema
Accumulation of edema fluid in spaces
in the outer plexiform and inner
nuclear layers
Complications of Uveitis
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Optic atrophy
Complications of Uveitis
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Hypotony
Due to proliferation of exudates in the
ciliary body
Diagnosis
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Differential-based
Diagnostic System
– Acquistion of facts
– Evaluate
– Listing of
Hypotheses
– Selection among
hypotheses
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Laboratory Testing
– Routine Tests
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CBC with differential
ESR/CRP
VDRL/FTA-ABS
– Frequently Obtained
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Chest X ray
ACE/lysozyme
ANA/RF/ANCA
Skin Testing
Diagnosis
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Specific Tests
– Blood Tests
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Autoantibodies
Specific serologies
Serum proteins
– Radiologic Tests
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Chest Radiography
Sacroiliac and
lumbosacral spine
Gallium scan
MRI
CT
Specific Tests
– FA
– Tissue Biopsy
– Ultrasonography
Treatment
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1.
2.
3.
Aims of therapy:
To prevent vision-threatening
complications
To relieve the patient’s discomfort
To treat the underlying cause
Treatment
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Four types of medications:
1) Mydriatics
2) Steroids
3) NSAIDs
4) Immunosuppressive medications
Specific Strategies for
Uveitis
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Type I: Eliminate agents, cromolyn,
antihistamine, corticosteroids
Type II: Dapsone, corticosteroids, cytotoxic
agents, surgical removal
Type III: NSAIDS, corticosteroids,
immunosuppressive and cytotoxic agents,
plasmapheresis
Type IV: corticosteroids,
immunosuppressive and cytotoxic agents,
cyclosporine
Treatment
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1) Mydriatics
To give comfort by relieving spasm of
the ciliary muscle
Atropine 1%, Tropicamide,
Cyclopentolate
To prevent formation of posterior
synechiae
To break synechiae
Treatment
2) Steroids
a) Topical steroids: only for anterior uveitis
 Prednisone, prednisolone, dexamethasone
 Usually start with a high rate of instillation
and decrease as inflammation lessens
 Complications: glaucoma, posterior
subcapsular cataract, corneal complications,
systemic side effects
Treatment
2) Steroids (cont)
b) Periocular injections
 Able to reach therapeutic
concentration behind the lens
 Longer lasting effect with depot
 Triamcinolone acetonide 40mg/ml
Methylprednisolone acetate 80 mg/ml
Treatment
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1)
2)
3)
4)
5)
Indications for periocular steroids:
Severe anterior uveitis
Resistant cases of anterior uveitis
Intermediate uveitis
Poor patient compliance
Cystoid macular edema
Treatment
2) Steroids (cont)
c) Systemic steroids (oral or
intravenous)
Corticosteroids
Adverse Reactions
Cushingoid changes (moon facies, weight
gain, fat redistribution, and increased
acne)
Delay of pubertal growth in children below
15
Infection, hypertension, fluid retention,
diabetes mellitus, hyperlipidemia,
atherosclerosis, osteoporosis, glaucoma
and cataracts
Anxiety, sleeplessness, mood changes,
easy bruising, poor wound healing
Pancreatitis, aseptic necrosis of bone,
myopathy, psychosis
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2) Steroids (cont)
d) Intravitreal steroids
Treatment
3) Non-Steroidal Anti-Inflammatory
agents (NSAIDs)
Flurbiprofen – effective in CME
Treatment
4) Immunosuppressive Drugs
Indications:
 When systemic corticosteroids are
insufficient to control the disease
 Steroid side effects
 Long term use of steroids at a high
dose to suppress the inflammation
Immunosuppressive
Drugs
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Antimetabolites
Azathioprine (Imuran)
Methotrexate (Rheumatrex)
Mycophenolate mofetil (Cellcept)
T-cell inhibitors
Cyclosporine (Sandimmune; Neoral)
Tacrolimus (Prograf)
Alkylating agents
Cyclophosphamide (Cytoxan)
Chlorambucil (Leukeran)
Common Ophthalmic Indications
for Cytotoxic Immunosuppression
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Type I: Autoimmune diseases: few
Type II: OCP, severe lens-induced uveitis
Type III: Autoimmune/collagen vascular
associated disease: Bechet’s disease, PAN,
Wegener’s granulomatosis, SLE, rheumatoid
arthritis
Type IV: Autoimmune diseases: SO, VKH,
birdshot retinochoirodopathy, pars planitis
Immunosuppressive
Usage & Side Effects
DRUG
SIDE EFFECT
Methotrexate
Liver damage &
mucosal lesions
Cyclophosphomide
Bone Marrow,
bladder, lung
CHOICE OF DRUG
FOR
Reiter’s Syndrome &
Severe Rheumatoid
Arthritis
OCP, Behcet’s, PAN,
relapsing
polychondritis,
Wegener’s
granulomatosis,
VKH, Serpigenous
chorioretinopathy,
SO
Immunosuppressive
Usage & Side Effects
DRUG
SIDE EFFECT
Azothioprine
Blood dyscrasia
Methotrexate
Mucosal ulcerations,
GI disturbances,
hepato & myelo
toxicity, infection
Nephrotoxicity
Cyclosporin
CHOICE OF DRUG
FOR
Wegener’s, OCP, JRA
Rheumatoid scleritis,
Ankylosing, Reiter’s,
SLE, JRA-associated
iridocyclitis
Bilateral sight
threatening
conditions: Birdshot,
SO, VKH, Behcet’s
Serpigenous
Malignancies of the Uveal
Tract
Sclera
Dense connective tissue that encloses about
five-sixths of the eye
- Functions:
 Strength and firmness maintains the shape
of the globe
 Aids in the maintenance of intraocular
pressure
 Provides attachment for the extraocular
muscles
 Protects intraocular structures from trauma
and mechanical displacement
-
Sclera
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1)
2)
3)
Layers of the Sclera
Episclera
Scleral Stroma
Lamina fusca
Sclera
1)
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-
-
Episclera
forms the superficial aspect of the sclera
and merges with the underlying scleral
stroma
Consists of loosely arranged bundles of
collagen, intermingled with fibroblasts,
occassional melanocytes, proteoglycans,
and gylcoproteins.
Rich blood supply mainly from the anterior
ciliary arteries
Sclera
Episcleritis
- a benign inflammatory disease
characterized by edema and cellular
infiltration of the episcleral tissue
- self-limited; recurrent; rarely leaves
any residual ocular damage
- Idiopathic in 2/3 of cases
Sclera
Episcleritis
- main symptom is mild discomfort
- Main sign is redness which may be
localized or involve the whole episclera
Sclera
2) Scleral Stroma
- consists of collagen bundles associated
with few elastic fibers
3) Lamina Fusca
- portion of the sclera adjacent to the
uvea
Sclera
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-
-
-
Scleritis
Severe inflammatory condition characterized by
edema and inflammatory cell infiltration of the
sclera
May be progressively destructive with loss of vision
Pain, tender globe, deep violaceous discoloration of
the globe due to dilation of deep vascular plexus of
the sclera and episclera
Associated with systemic disease
Sclera
Clinical classification of episcleral and scleral
inflammation
Episcleritis
Simple
Nodular
Scleritis
Anterior scleritis
Diffuse scleritis
Nodular scleritis
Necrotizing scleritis
Posterior scleritis
Sclera
Disease associated with episcleritis and scleritis
Diagnosis
% of patients with associated
disease
Episcleritis
31.91%
Simple
28.21%
Nodular
50.00%
Scleritis
56.98%
Diffuse
45.45%
Nodular
43.59%
Necrotizing
94.87%
Posterior
45.45%
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