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Julie Jantzi, O.D.
Mallory Cranmer, O.D.
Neal Shastri, O.D.
Noushin Ahmed, O.D.
Seidenberg Protzko Eye Associates
Epidemiology
• ~15 per 100,000 in U.S. each year
• Causes 10% of blindness in U.S.
• Third leading cause of blindness in developed countries
• Prevalence varies by location, age, and date of study
• Highest among >65 years of age
• Females
• Chronic and unilateral
• Anterior Uveitis most common
Pathophysiology
• Inflammation of the uvea
• Infectious, traumatic,
neoplastic, autoimmune,
idiopathic
• Inflammatory response:
chemical mediators result
in vasodilation, increased
vascular permeability, and
chemotaxis of
inflammatory cells in eye.
Inflammatory Cascade

Photo Credit: Studyblue.net
Classification
Location

Duration

Type

Anterior Uveitis

Acute

Granulomatous

Intermediate
Uveitis

Chronic

Nongranulomatous

Posterior Uveitis

Panuveitis
Symptoms
• Redness
• Pain
• Photophobia
• Epiphora
• Blur
• Scotoma
• Floaters
Photo Credit: www.sunglasswarehouse.com
Clinical Signs
Anterior to Posterior
Eyelid
• Vitiligo
• Nodules
• Lesions

Photo credit: http://www.pcds.org.uk/clinical-guidance/vitiligo
Conjunctiva & Episclera
• Ciliary Flush

• Diffuse injection

Photo credit: http://www.mastereyeassociates.com/eyediseases-treatments/iritis---anterior-uveitis/
Cornea
• Keratic Precipitates
• Granulomatous
• Non-granulomatous

• Fibrin
• Pigment
• Corneal Edema
• Band Keratopathy*
Photo Credit:
http://www.optometricmanagement.com/articleviewer.aspx?artic
leid=71765
Anterior Chamber
• Cells
• Flare (serous)
• Pigment
• Hypopyon (purulent)
• Plasmoid (Fibrinous)
• Hyphema + hypopyon
(sanguinoid)
Grading
Iris
• Nodules
• Busacca
• Koeppe

• Posterior synechiae
• Heterochromia
• Granulomas
• Atrophy (stromal)*
• Cyclitic membrane
formation*
Angle
• Nodule (Berlin)
• Vascularization
• Peripheral anterior
synechiae

• Secondary Ciliary body
detachment*
IOP
• Hypotony
• Secondary Glaucoma*

(Van Bus-kirk EM: Clinical Atlas of Glaucoma. Philadelphia: WB Saunders, 1986.)
Vitreous
• Cells
• Flare
• Snowball Opacities
• Snowbanking
• Active
• Inactive

• Vitreal Strands

Photo credit: Mohawk Valley Retina
Retina
•

Retinal/choroidal
inflammatory infiltrates

•

Sheathing of arteries/veins

•

Retinal detachment

•

Schwartz syndrome

•

RPE hypertrophy/atrophy

•

Atrophy/swelling of retina,
choroid, optic nerve head

•

Macular edema

Photo Credit: Oculist.net
Major Complications
• Cataracts
• Glaucoma
• Band Keratopathy
• Cystoid Macular Edema
Differential Diagnoses
•

Infections

•

Non-infectious

•

Masquerade syndromes

•

Large cell Lymphoma, retinoblastoma, leukemia, malignant melanoma

•

Juvenile Xanthogranuloma

•

Pigment dispersion syndrome

•

Retinal Detachment

•

Retinitis pigmentosa

•

Ocular Ishchemic Syndrome
Non-Infectious Uveitis
ANTERIOR UVEITIS:

POSTERIOR UVEITIS:

•

•

Collagen Vascular Disease

•

White Dot Syndromes

•

Acute nongranulomatous Iritis
and Iridocyclitis
Chronic Anterior Uveitis

PANUVEITIS
INTRMEDIATE UVEITIS:
•
•

•
•

Sarcoidosis
Sympathetic Ophthalmia

•

Vogt-Koyanagi-Harada Syndrome

•

Behcet Disease

Pars Planitis
Multiple Sclerosis
Acute Anterior Uveitis
HLA-B27 diseases
Glaucomatocyclitic crisis
Lens associated uveitiis
Postoperative inflammation: IOL associated
HLA-B27 DISEASES
•

Class 1 surface antigen

•

Seronegative spondyloarthropathies are strongly associated with acute
anterior uveitis and HLA-B27

•

spondylitis and sacroiliitis

Include:

•

Ankylosing spondylitis

•

Reactive arthritis syndrome

•

Inflammatory bowel disease

•

Psoriatic arthritis
Ankylosing
Spondylitis
•

lower back pain and
stiffness

•

men, 20-40‟s

•

HLA-B27: 90%

•

dx: sacroiliac imaging
studies - “bamboo spine”

Ocular findings (40%):
•

acute anterior uveitis - fibrin
and hypopon not
uncommon
Reactive Arthritis Syndrome

•

Triad: nonspecific urethritis,
polyarthritis, and conjunctivitis
often accompanied by iritis

•

HLA-B27: 95%, young adult men

•

arthritis in knees, ankles, feet,
sacroiliitis in 70% of pts

Ocular findings:
•

Mucopurulent and papillary
conjunctivits, acute
nongranulomatous iritis in 10%
Keratoderma blennorrhagicum
Psoriatic Arthritis
•

Inflammatory arthritis occurs in 30% of
pts with chronic psoriasis

Inflammatory Bowel Disease

•

cutaneous changes, distal phalangeal
joint inflammation, ungula involvement

•

•

20% may have sacroiliitis

•

25% develop iritis

12% of ulcerative colitis pts
and 2.4% of Chrons
disease pts develop acute
anterior

•

20% have sacroiliitis

•

60% HLA-B27 positive
Glaucomatocyclitic Crisis
•

Recurrent unilateral mild
acute iritis

•

Elevated IOP, corneal edema,
fine KPs, low-grade cell and
flare, slightly dilated pupil

•

Duration: hours to days

•

Associated with HLA-B54
Lens-associated
Uveitis
•

•

Immune reaction to lens
material: granulomatous or
nongranulmatous
KPs can be small or large,
cells mild to severe, hypopon,
PS, elevated IOP, vitritis

Postoperative
inflammation:
IOL-associated
Mild inflammation to UGH syndrome
(uveitis-glaucoma-hyphema)
IOL implantation can activate
complement cascade, cellular deposits
on IOL, synechiae formation, capsular
opacification

•

Retained lens material

•

Phthisis bulbi, persistent uveitis,
or glaucoma often occurs in
these eyes

•

iris chafing caused by the edges or
loops of IOL – mechanical irritation and
inflammation
Drug Induced Uveitis
•

Systemic Drugs

1. Rifabutin
2. Bisphosphonates

•

Topical Drugs

1. Metipranolol
2. Miotics
3. Prostaglandins

3. Sulphonamides
4. Diethylcarbamazine

•

Intracameral Drugs

1. Cidofovir

5. Cidofovir (intravenous)
2. Antibiotics
3. Urokinase

•

Vaccines
Chronic Anterior
Uveitis
Juvenile idiopathic arthritis
Fuchs heterochromic iridocyclitis
Idiopathic iridocyclitis
•

most common systemic disorder
associated with iridocyclitis in
children

•

iritis develops within 5-7 yrs of
onset of joint disease

•

RF: female, pauciarticular onset,
ANA

•

negative RF

•

eye is usually white and uninflamed, some pts do not have
pain

•

Ocular findings: fine KPS, flare and
cells, posterior synechiae, cataract,
glaucoma, band keratopathy,
vitreous debris, ME, chronic
hypotony, phthisis

Juvenile Idiopathic
Arthritis
Fuchs
Heterochromic
Iridocyclitis
•

Unilateral uveitis,
heterochromia, iris stromal
atrophy, small white stellate
KPs scattered diffusely over
endothelium

•

synechiae almost never
forms but glaucoma and
cataracts often occur

•

Associations with
toxoplamosis, HSV, and
CMV infections
Idiopathic Iridocyclitis
•

In many cases of chronic iridocyclitis the cause is unknown
Intermediate Uveitis
Pars Planitis
Multiple Sclerosis
•

absence of infection and systemic
disease

•

85-90% of intermediate uveitis
cases

•

5-40 yrs, healthy individuals

•

associated with HLA-DR15 and HLADR51 alleles

Pars Planitis

Clinical Characteristics:

•

80% bilateral

•

Ocular manifestations: spillover into
anterior chamber, vitreous cells,
snowballs, peripheral phlebitis and
retinal venous sheathing

•

Ddx: Syphilis, lyme, sarcoidosis, MS,
toxicarasis, lymphoma
Multiple Sclerosis
•

30% of MS pts will get
uveitis

•

15% of pts with pars planitis
will eventually develop MS

•

women, 20-50 yrs

•

HLA-DR2 and DR15

•

Granulomatous anterior
uveitis, intermediate uveitis,
panuveitis
Posterior Uveitis
Collagen Vascular Diseases
Inflammatory Chorioretinopathies of Unknown Etiology
Collagen Vascular Disorders
Systemic Lupus Erythematosus
Polyarteritis Nodosa and Microscopic polyangiitis
Wegener‟s Granulomatosis
Systemic Lupus
Erythematosus

•

Multisystem autoimmune
connective tissue disorder

•

Type 3 hypersensitivity reaction:
Antibody-immune complexes
precipitate and cause further
immune response

•

Affects heart, nervous system,
joints, skin, lungs, blood vessels,
liver, and kidney

•

Women, 15-45 years

•

Labs: ANA

•

Malar “butterfly” rash -70-80%
Clinical findings
Ocular manifestations in 50%

•

Keratoconjunctivitis sicca
(20%)

•

Lupus retinopathy -CWS

•

Cutaneous lesions on the
eyelids

•

Scleral inflammatory
disease

•

Neuro-ophthalmic disorders

•

Retinal vasculopathy

•

In rare cases uveitis
Polyarteritis nodosa
and microscopic
polyangiitis
•

Systemic vasculitis –
necrotizing inflammation of
medium and small sized
arteries

•

40-60 yrs, men, hepatitis B

•

Ocular involvement in 20%

•

Mortality rate of untreated
PAN is 90% by 5 yrs

•

Labs: p-ANCA, ESR, CRP,
tissue biopsy
Wegener‟s
Granulomatosis
•

Triad: necrotizing granulomatous vasculitis of upper and lower respiratory
tract, glomerulonephritis, necrotizing vasculitis of small arteries and veins

•

Involvement of paranasal sinuses is most characteristic feature

•

Ocular involvement in 50%:

-- orbital involvement: pseudotumor, cellulitis, dacryocystitis
-- anterior, intermediate, or posterior uveitis
--scleritis (necrotizing), uveitis, retinal vascular involvement
Dx: tissue biopsy, chest x-ray, CRP, ESR, c-ANCA
White Dot Syndromes
• Group of chorioretinopathies that are inflammatory in
nature presenting with discrete, multiple, yellow-white
lesions
• Symptoms: photopsia, blurred vision, floaters,
nyctalopia, enlarged blind spot
• Young females
•

Acute posterior multifocal placoid
pigment epitheliopathy (APMPPE)

•

Birdshot choroidopathy

•

Multiple evanescent white dot
syndrome (MEWDS)

•

Acute zonal occult outer retinopathy
(AZOOR)

•

Multifocal choroiditis (MCP)

•

Punctate inner choroidopathy (PIC)

•

Serpiginous choroiditis
Panuveitis
Sarcoidosis
Sympathetic Ophthalmia
Vogt Koyanagi-Harada Syndrome
Behcet Disease
Sarcoidoses
•

Multisystem non-caseating
granulomatous disorder of
unknown etiology

•

Intrathoracic : 90%, intraocular:
50%

•

Affect lymph nodes, skin, eyes,
CNS, bones and joints, liver, and
heart

•

African American, females, 20-50
years

•

Dx: chest x-ray, lymph node biopsy,
elevated ACE and lysozyme levels
Ocular Findings
•

can involve any ocular tissue

•

most common: Anterior
granulomatous uveitis

–mutton fat KP‟s, koeppe and
busacca iris nodules, synechiae

•

Posterior segment in 20% of
ocular sarcoidosis

--vitreous infiltration “string of
pearls” or “snow balls”
--granulomas seen in retinal,
choroid
--perivascular sheathing,
occlusive vascular disease
•

Eyelid granulomas

•

Conjunctival nodules

•

Lacrimal gland infiltration
Sympathetic Ophthalmia
•

Bilateral, diffuse granulomatous, non-necrotizing
panuveitis

•

Autoimmune inflammatory response toward ocular
antigens – delayed hypersensitivity to melanin
containing structures?

•

Occurs after surgery or trauma to an eye

•

2% of uveitis cases

•

Anterior Uveitis: mutton fat KPs, PS, thickening of
iris from lymphocytic infiltration

•

Posterior vitritis, dalen-fuchs nodules,
peripapillary choroidal lesions, exudative RD
•

Cell-mediated autoimmune process
driven by T lymphocyctes directed
against self-antigens associated with
melanocytes of all organ systems

•

Higher association in darkly pigmented
ethnic groups

•

4% of uveitis referrals in US

•

Chronic, bilateral, diffuse,
granulomatous panuveitis with
integumentary, neurologic, and auditory
involvement

Vogt-KoyanagiHarada Syndrome
Stages
Acute: bilateral granulomatous
anterior uveitis, vitritis,
thickening of choroid, edema
and hyperemia of optic nerve,
multiple serous retinal
detachments

Convalescent: “sunset glow”
fundus, skin changes: vitiligo,
alopecia, poliosis

Chronic recurrent: repeated
bouts of uveitis
Behcet Disease
• chronic, relapsing,
occlusive systemic
nongranulomatous
vasculitis of unknown
etiology

• recurrent oral ulcers,
skin lesions, genital
ulcers, ocular
inflammatory disease
(70%)
• can affect all portions
of uveal tract
Ocular Findings
80% bilateral
• Anterior uveitis:
hypopyon in 25% of
cases
• Posterior segment:
necrotizing retinal
vasculitis affects both
veins and arteries

• Optic nerve affected in
25% of pts
• Visual prognosis is
guarded -25% VA <
20/200
Infectious Uveitis
•Viral – Herpetic, CMV
•Fungal
•Protozoal - Toxoplasmosis
•Helminthic - Toxocara
•Bacterial – Syphilis, Lyme, Tuberculosis, other
Viral

• Herpes Simplex:
• Usually iritis is a keratouveitis but can be: corneal,
cutaneous, retinal
• arborized

• +/- KPs, +/- Hypopyon
• HSV-1 is generally oral-labial HSV-2 generally genital
infections, although crossover does occur.
Image: C. Blake Perry. Source: http://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/HSV-epithelial-keratitis.htm
Viral

• Herpes Zoster
•
•
•
•

72 hours PHN
¼ ocular involvement
Infiltrative pseudodendrites (negative stain)
Typically elderly and immunocompromised

Image:Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 223. Print.
•

Viral
ARN
•
•
•
•
•
•

VZV>HSV>CMV
Immunocompetent
Starts unilateral but 36%Fellow eye
Within 6 weeks of onset
Painful, Floaters, LOV
Peripheral „creamy‟ necrosis that spread,
occlusive vascular involvement, Panuveitis
• Poor Prognosis, RD in 75%

• PORN
• VZV mainly
• Immunocompromised
• Posterior pole involved early
• Patchy confluent necrosis, weak or
absent uveitis, no vascular involvement
• Poor Prognosis, RD in 70%
Images: Baumal, Caroline et al. Duane‟s Ophthalmology ,2006.‟Acute Retinal
Necrosis and Progresive Outer Retinal Necrosis Syndromes Accessed online:
http://www.oculist.net/downaton502/prof/ebook/duanes/pages/v3/v3c028.html#ref
Viral
• CMV
•
•
•
•
•
•
•

CD4 <50-100
Most common opportunistic infection in AIDS
In congenital cases + birth defects
Before HAART ~30% of CD4<50
After HAART incidence decreased 75-80%
May start by looking like CWS (small infiltrates)
Perivascular hemorrhages, white necrotic retina, frosted
branches /vasculitis

Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 796. Print.
Protozoal
• Toxoplasmosis
• Toxoplasma gondii
• Undercooked meat /cat feces
• Most common posterior uveitis in immunocompetent
individuals
• Often acquired in utero (0.2-1% of pregnancies)
• Further Trimester = increased chance of exposure, worst if
acquired early (spontaneous abortion)
• Majority do not develop ocular manifestations
Image: Tabbara. Khalid. „Toxoplasmosis‟. Duane‟s Ophthalmology 2006. Accessed online:
http://www.oculist.net/downaton502/prof/ebook/duanes/pages/v4/v4c046.html
Protozoal
• Toxoplasmosis
• „Headlights in the fog‟
• Focal chorioretinal scar with adjacent necrosis and dense
vitritis
• With AIDS may have multiple lesions
• Often self-resolving over months
• May use sulfadiazine, pyramethamine, steroids.
Image: Tabbara. Khalid. „Toxoplasmosis‟. Duane‟s Ophthalmology 2006. Accessed online:
http://www.oculist.net/downaton502/prof/ebook/duanes/pages/v4/v4c046.html
Helminthic Parasites
• Toxocaraisis
• Ingesting eggs of Toxocara
• Dogs >cats
• Acquired through soil, or uncooked food
• Particularly affects kids with pica

• Yellow white mass with tractional component, may have
vitritis.
• Often leads to TRD
• Treat uveitis, consider anti-helminthics (albendazole)
Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 834. Print.
Bacterial
• Syphilis
•
•
•
•
•
•

Treponema pallidum
„Great Mimicker/Masquerader/Imitator‟
Enters through intact mucosa
Ocular Syphilis in 2‟ and 3‟ stages (2-6 mo after)
Uveitis most common ocular manifestation (up to 10%)
Affects immunocompetent or immunocompromised

Image: „Syphilis‟. WebMD. Accessed Online: http://www.webmd.com/sexual-conditions/guide/syphilis
Bacterial
• Syphilis
•
•
•
•
•
•
•

Dilated iris capillaries (iris roseola)
Chorioretinitis, „salt and pepper‟
Vasculitis, vitritis, neuroretinitis, CNV, optic neuritis
Granulomatous or non-granulomatous uveitis
Argyll Robertson
Interstitial Keratitis
Tests:
• Nontreponemal (VDRL / RPR)
• Treponemal (FTA-ABS / MHA- TP)

• Tx: Penicillin
Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 800. Print.
Bacterial
• Lyme

•
•
•
•
•

Borrelia burgdorferi
1: rash (e. migrans)
2: dermatologic, neurologic, cardiac
3: Late arthritis, cranial and peripheral nerves
50% reported no hx of tick bite

Image: Sources for Rashes: Poster: "Looking for a Bull's-Eye Rash? Look again - erythema migrans can take many
forms. A collaborative effort between the Maryland Department of Health and Mental Hygeine and the Lyme
Disease Research Foundation of Maryland Maryland.http://www.bayarealyme.org/get-help/lymes-many-symptoms
Bacterial
• Lyme

1. Conjunctivitis early
2.Motility / CN palsies, optic neuropathy, PTC,
chorioretinitis, uveitis
3. Stromal keratitis, symblepharon
ELISA, Western Immuoblot
Tx: Doxycycline, amoxicillin, if needed ceftriaxone
Image: Pediatric Associates of Connecticut. „Tick Bites and Lyme Disease, Accessed Online:
http://www.pediatricdoc.com/newsletter.cfm?nid=625
Bacterial
• Tuberculosis
•
•
•
•
•
•
•
•
•
•

Mycobacterium tuberculosis
Incidence: Annually ~9.3 million patients worldwide
Communicable: inhaled aerosolized droplets
Primarily involves the lungs
Uveitis is common
Cough, fever, night sweats
Caseating necrotic granulomas
CXR
PPD / Mantoux testing
TB blood tests: interferon-gamma release assays (IGRAs)

Images taken from wikipedia . Accessed online: http://en.wikipedia.org/wiki/Tuberculosis_diagnosis
Bacterial
• Tuberculosis

•
•
•
•

Granulomatous anterior, intermediate or posterior uveitis
Vasculitis with choroiditis, or choroidal tubercles
Phlyctenular conjunctivitis
Tx: Rifampin, Isoniazid, Pyrazinamide, Ethambutol

Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 808. Print.
Infectious
Endophthalmitis
• Endogenous (<10%)
• Systemic via blood stream
• Organisms causing endocarditis and GI tract are primary sources,
usually with sepsis, immunocompromise, IV drug abuse,
catheders.
• Typically less pain and inflammation, and can be bilateral

• Exogenous
•
•
•
•

Trauma, Surgery, Infection
Acutely is usually gram + (normal flora)
Decrease in occurrence over the last 30 years.
Bleb-associated may occur at any time.
• Acute 0.061-0.3%
• Delayed 0.2-18% (variable reports)

Image taken from „Endophthalmitis‟ by the American Academy of Ophthalmology 2013. Accessed Online:
http://www.aao.org/theeyeshaveit/red-eye/endophthalmitis.cfm
Infectious
Endophthalmitis
• EVS
• used <6 weeks, for cataract PO (Acute Onset)
• Blur (94%), red eye (82%), pain (74%), hypopyon
(86%)
• No view of retinal vessels in 79%
• Red reflex present in only 32%
• Immediate vitrectomy if LP or worse.

• B scan
• Tx: culture, Vancomycin/Amikacin/Ceftazidime +/Dexamethasone
Masquerade
Syndromes
Accounts for nearly 5% of all patients with uveitis at tertiary referral.
Neoplastic Masquerade
• Lymphoma
• Uveal melanoma
• Retinoblastoma
• Juvenile xanthrogranuloma
• Metastatic tumors

Nonneoplastic Masquerade
• Retinitis Pigmentosa
• Ocular Ischemic Syndrome
• Retinal detachment
• Intraocular Foreign Bodies
• Pigment Dispersion Syndrome
Lymphoma
• 5th to 7th decade of life
• 25% ocular involvement
• 15% ocular as initial presentation
• 56% with ocular involvement develop
CNS involvement

• Variable uveitic presentations
unresponsive to therapy
• Can mimic chronic uveitis
Lymphoma: Ocular Findings
• Benign reactive uveal
lymphoid hyperplasia
• Mobile, fleshy
episcleral/conj mass
• Retinal findings: classic
creamy-yellow sub-retinal
infiltrates with overlying
RPE detachments
mimicking sarcoid or
birdshot

• Anterior uveitis
• CME
Lymphoma
• Diagnosis
• MRI
• Cerebrospinal fluid
analysis
• Tissue diagnosis
(definitive method)

• Treatment
• Intravenous and
intravitreal methotrexate
• Radiation & IV
Cytarabine
• >60 years = chemo only
• <60 years = combination
radiation & chemo

• Prognosis = poor
Uveal Melanoma
• 5% ocular inflammation
• Episcleritis, ant/post
uveitis, endophthalmitis

Retinoblastoma
• 1-3 % with ocular
inflammation
• White pseudohypopyon
• Conjunctival chemosis

• Ultrasound: low internal
reflectivity

• Diffuse infiltrating variant

• 4-6 years old
• Limited visibility of fundus
Juvenile
Xanthogranuloma
• Occurs before age 1
• Red-yellow skin lesions
• Lesions can involve iris =
spontaneous hyphemas

• Skin of eyelid involved,
globe usually spared
• Treatment: topical,
periocular, systemic
steroids
Metastic Tumors
•

Adults

•

•

Metastic melanoma: brown
spherules in retina

Most common: Cutaneous
Melanoma

•

Others: white-yellow

•

Perivascular sheathing,
necrotizing retinitis, retinal
vasculitis

• Lung, GI, breast

•

Vitritis, serous detachment,
CME

•

Iris nodules, NVI, high IOP

•

Anterior uveitis

•

Bilateral and multifocal

•

Rare
Nonneoplastic
Masquerade
• RP
• Waxy disc pallor,
attenuation of arterioles,
bone-spicule mid-periphery
• +FHx
• Nyctolopia
• Vitritis, CME
• ERG depressed early
Source: http://www.rpfightingblindness.org.uk
Ocular Ischemic
Syndrome
• Males >65
• CC: decreased vision, mild
pain
• Corneal edema, A/C
reaction, flare>cells, NVI,
NVA,
• decreased IOP (ischemia),
high with NVG
• Mild disc edema, dilated
tortous veins
• mid peripheral hemes
• NVD/NVE
• FA
• Delayed arteriole filling
• Diffuse leakage
• Capillary non-perfusion

•

Carotid doppler > 90%
diagnostic

•

Treatment
• Endarterectomy
• Topical corticosteroids &
cycloplegics
• PRP for neo
• Anti-VEGF injection
• 5 year mortality rate of
patients 40%
• Transient improvement,
usually worsens
Retinal Detachment
• Chronic Peripheral
Rhegmatogenous
• AC reaction
• Vitreous inflammatory &
pigment cells
• Good vision, may worsen
due to CME

• Photoreceptor outer
segments may be present
in AC simulating
inflammatory response
• IOP elevated, OAG
• Schwartz syndrome

• DFE with scleral
depression
• Peripheral demarcation
lines, subretinal fluid,
retinal breaks, subretinal
fibrosis, peripheral cysts

http://gridironforgirls.blogspot.com
Intraocular FB
• Chronic inflammation
• Mechanical, chemical,
toxic, or inflammatory
inflammation of ciliary
body
• Gonio, ultrasound, CT of
eye/orbits
• Complications:
proliferative
vitreoretinopathy and
endophthalmitis

Pigment Dispersion Syndrome
• Pigment released from iris
and/or ciliary body

• Simulates anterior uveitis
Management and
Treatment
Cycloplegics and mydriatics
Corticosteriods
Immunomodulating Agents
Pressure-lowering medications
Cycloplegics
Mydriatics

• These opthalmic
preparations block the
responses of the iris
sphincter and the
accommodative muscle of
the ciliary body to
cholinergic stimulation,
producing pupillary dilation
(mydriasis) and paralysis of
accommodation
(cycloplegia)
• Tropicamide 1%,
cyclopentolate and atropine
have both properties
• Phenylephrine has only
mydriasis.
www.lookfordiagnosis.com/mesh_info.php?term=
Cycloplegics
Purpose:
•To break or prevent posterior
synechiae and to relieve ciliary
spasm induced photophobia.
•Short acting vs. long acting
cycloplegics
•Dosing depends on the extent
of inflammation
•(1-2xdaily atropine, 3xdaily
tropicamide and
phenylephrine)
www.opsweb.org/?page=Scien
tificExhibit
portalcodgdh.minsaude.pt/index.php/Dissecção
_

Posterior synechiae
Antigen-dependent lymphocyte activation and STEROID/NSAID
activation sites

Cunningham, Emmett. Practical approach to the use of corticosteroids in patients
with uveitis. Can J Opthalmol 2010; 45:352-8
Corticosteroids:
Produced through the
hypothalamus, pituitary and adrenal axis
(HPA) to control inflammation in uveitis

http://pubs.niaaa.nih.gov/publications/arcr
344/images/stephens01.png
Common uses of
corticosteroids
-Mainstay of initial therapy

Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with
uveitis. Can J Opthalmol 2010; 45:352-8
Ocular Surface
Penetration
Durezol>Pred
Forte> FML
larrazabaleye.com/cvep.html
www.revoptom.com/content/c
/216
www.revoptom.com/content/c
/216

TOPICAL STEROIDS
Topical Steroids

Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with uveitis. Can
J Opthalmol 2010; 45:352-8
Topical Steroids

•Suspension vs.
emulsion
•Concentration vs.
efficacy
www.pharmainfo.net/reviews/microemulsionsnovel
Potency

Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with
uveitis. Can J Opthalmol 2010; 45:352-8
Adverse Effects of
Corticosteroids
Adverse Effects continued

www.amardeepeyecare.com/ptosis.html
continued

www.lpeyecare.com/eyeconditions/252-2
Intravitreal Injections

Cunningham, Emmett. Practical approach to the use of corticosteroids in
patients with uveitis. Can J Opthalmol 2010; 45:352-8
NSAIDS

•

www.uninet.edu/cin2001-old/conf/bala/bala.html

•

www.thedrugmonitor.com/coxi.html
Systemic NSAIDS
• Complications of prolonged use:
-myocardial infarction
-hypertension

-stroke (selective Cox-2 inhibitors)

www.mayoclinic.com/health/medic
www.ohioinjurylaw.com/ohioattorney/medical-mal
Immunomodulating
Medications (IMT)

• Antimetabolites

• T cell signaling Inhibitors
• Alkylating Agents
• Biologic response modifiers

www.riskindoc.com/new_psoria
Indications for IMT
 inadequate response of uveitis to corticosteroid therapy for greater than 3
months with doses exceeding 5-10mg/day.
 contraindications to corticosteroid use:
1. (diabetes, hypertension, peptic ulcer, GERD, immunocompromised
state, psychiatric conditions)

2. exacerbated side effects
3.chronic corticosteroid dependence

www.fortiusfitness.com/blog/steroi
d-side-effects
Precautions
 No infection present
 No hepatic or hematologic contraindications
 Close physician follow up
 Teratogenic

mamaprayed.blogspot.com/2012/08/if-you-give-m
Antimetabolites
•

Azathioprine, methotrexate, mycophenolate mofetil

•

Azathioprine (purine nucleoside analogue)

-interferes with DNA replication and RNA transcription
-found beneficial in VKHsyndrome, int. uveitis, Behcet uveitis, sympathetic
opthalmia and necrotizing scleritis
-GI side effects (nausea, upset stomach) are cause for D/C.

-CBC, LFT every 4-6 weeks.
-Thiopurine S-methyltransferase (TPMT) testing (metabolizes 6mercaptopurine,ie 6-MP)

www.lookfordiagnosis.com/mesh_info.php?term
Methotrexate
•

Folic Acid analogue

•

Inhibitor of dihydrofolate
reductase and it’s DNA
replication

•

Causes extracellular
accumulation of adenosine to
create an anti-inflammatory
effect.

•

Useful for JIA (first line choice
IMT treatment for
children), sarcoidosis, panuveiti
s, various other types of uveitis

•

Prospective study for IV inj. for
refractory uveitis and uveitic
CME.

www.humira.com/jia/what-is-jia.aspx
Mycophenolate Mofetil
• Inhibits inosine monophosphate dehydrogenase
and DNA replication
• Side effects: reversible GI distress, diarrhea;
20%pt. pop.
• CBC monthly
• 85% effectivity in chronic uveitis patients and a
good second choice IMT for children.
T Cell Signaling Inhibitors
Cyclosporine and tacrolimus:
•

Calcineurin inhibitors- disrupt
T-cell receptor signal
transduction and down regulate
IL-2 transcription and CD4 T
lymphocyte receptor expression.

Sirolimus:
•

Non-calcineurin inhibitordisrupts T cell signaling that
inhibits Ab production and Blymphocytes.
www.bdbiosciences.com/research/tcell/reg
Cyclosporine
• SE: Nephrotoxicity and systemic hypertension;
paresthesia,hypertrichosis.
• BP, CBC, serum creatinine monthly.
• Used for int. uveitis and posterior uveitis cases from
Behcets and VKH.
• Used in combination with corticosteroids for modest
improvement
Tacrolimus
• AE: nephrotoxicity

• Low dose and increased potency to cyclosporine
• Less risk of hypertension and hyperlipidemia
• Useful for chronic int. and posterior uveitis.
Sirolimus
• One open-label, prospective study it was found useful in
treatment for refractory noninfectious uveitis
• GI side effects
• Under active investigation for use in treating uveitis
Alkylating Agents
Cyclophosphamide
• Active metabolites alkylate purines in DNA and RNA, creating
impaired DNA replication and cell death
• Cytotoxic to dividing and resting lymphocytes
• AE:myelosuppression, hemorrhagic cystitis, sterility
Chlorambucil
• Interferes with DNA replication
• AE:myelosuppression, sterility

Treatment for int. uveitis, VKH, SO, Behcet
Significant AE: increased risk for malignancy (leukemia, various cancers)
Infliximab

Biologic Response
Modifiers

• Chimeric, monoclonal IgG1k antibody against TNFalpha
• Treatment for Behcet, sarcoidosis and VKH. Some HLA
B27 ant. uveitis
• Drug induced toxicity (lupus, systemic vascular
thrombosis, CHF, malignancy, demyelinating disease and
vitreous hemorrhage)
• +PPD, contraindication
• Adalimumab, less AE, same effectiveness
Alternative Therapy Under
FDA Investigation
• Rituximab:chimeric monoclonal antibody against CD20
positive cells (B lymphocytes)
• Daclizumab: monoclonal Ab to IL-2
• IFNalpha2a: antiviral, immunomodulatory and
antiangiogenic effects (leukopenia and thrombocytopenia
side effects)
Management/Follow up
• Encounter visit determines
extent of ocular inflammation
(ant., int., panuveitis, etc).
• Make a differential list and
send out for further pertinent
lab work to confirm/rule out
differentials.

super-trainer.com/fitness-business-becoming-a-bu
Anterior Uveitis
• Cycloplegic
• Steroid
• Dosage depends on severity
• Follow up in 2-3 days
• If 2-3 additional follow up
appt. prove no
improvement, referral to
uveitis specialist

medlibes.com/entry/anterior-uveitis
Intermediate/Posterior Uveitis
• Determine extent of
inflammation, if anterior
involvement, start on
cycloplegic and steroid.
• Refer to retinal specialist for
periocular steroid injections (if
deemed non-infectious)
• Intravitreal injections for
chronic anterior uveitis or
inflammation not responsive
to periocular injection
www.retinaeye.com/uveitis.html
Uveitic Treatment
Adverse Effects
• Steroid responders:
Need to lower IOP by utilizing beta blockers, alpha2
agonists,CAI, prostaglandins and their combinations
where appropriate.
Case #1
• 70+ yr old cauc. male
• Redness, mild pain, blurry
vision in the right eye.
• Slit lamp: 2+ AC WBC
reaction; florrid NVI,NVA,
corneal edema with descemet
folds; IOP 16mmHg OD
Differentials?
• Neovascular glaucoma

• OIS
• PDR
OIS!
• DFE: mid-peripheral blot hemorrhages
• No CME, NVD, NVE
• Tx: Prednisolone Forte q2hrs, Atropine BID OD
• Referral to retinal consult for PRP and possible injections of anti-vegf

• Contact PCP for carotid doppler, echocardiogram, EKG
Case #2
• 17 yr cauc. female
• CC:
redness, pain, photophobia, bl
urry vision
• Review of systems was
unremarkable
• Second episode, first episode
two years ago in GA.
Review of Systems
• General - Weight loss, fatigue, fever Skin - Rash, nodules, changes in
nails
• Neck - Lymphadenopathy
• Respiratory - Cough, wheezing
• Cardiac - Chest pain/discomfort, dyspnea
• Gastrointestinal - Hepatomegaly, diarrhea, frequent bowel movements
• Genitourinary - Delayed secondary sexual characteristics
• Musculoskeletal - Muscle or joint pain, arthritis, back pain, limitation
of motion
(refer to uveitis.org for more information and questionnaire)
Differentials?
• JIA, lyme disease, lupus, sarcoid
• ?????
• Ran pertinent lab tests for her age (CBC c diff.,ANA, ACE,
Lyme titer,ESR,RF, HLA B27)
• Durezol q2hrs, Atropine BID OD. Pledget of 10%
phenylephrine to break posterior synechiae
• CBC with diff., lyme titre, RF, ANA, ACE, serum lysozyme
• Blood results…negative. Consult with uveitis specialist.
Case 3
Chief Complaint
• 46 year old WM
• Redness OS x 5 days, gradually getting worse
that is constant throughout the day
• (+) photophobia, epiphora
• ( - ) eye pain, flashes, floaters, cold sores

• He was seen at Patient First 3 days ago where
they diagnosed him with shingles over the left
eyelid, noting ocular involvement

• Treated with 20 mg Pred, mild pred taper and 1
gm of Valtrex tid x 10 days
History
• Medical History
• Hypertension, GERD
• Treated with Omeprazole and Diovan

• Family Medical History
• Unremarkable
Examination
• VA sc 20/25 OD, OS, OU
• OD unremarkable findings
• OS
• Lid: vesicles along upper
forehead & nose, lid
erythema, small hordeolum
• Conj: nasal injection
Examination
• Cornea
• Punctate Epithelial Keratitis
• Inferior stromal haze

• Anterior Chamber
• Cells – WBC 2+
• ( - ) Flare

• IOP 16, 19 mm Hg
• Fundus
• Unremarkable OU
Examination
• Fundus Unremarkable, OU
Assessment
1. Redness/Discharge of
Eye OS

1. Educated patient on
findings

2. H. Zoster
Keratoconjunctivitis OS

2. Neomycin ung on
vesicles, BID

3. H. Zoster Iridocyclitis, OS

3. Pred Forte TID, OS

4. H. Zoster of Eyelid

4. Cool compresses and
antibiotic ung on vesicles
Follow Up: 2 days later
• Patient now complains
of eye pain in the
morning

• Thinks it is getting
worse
• Currently taking pred
forte tid OS and will
finish Valtrex the
following week
Examination: 2 days
•

VA sc 20/25 OD; 20/40 OS PHNI

•

OD unremarkable

•

OS Lid: Vesicles along upper
forehead & nose with lid
erythema, hordeolum on superior
lid

•

Cornea
• Prominent pseudodendrites

•

Anterior Chamber
• 2+ WBC

•

IOP 16, 27

•

Fundus: Unremarkable OU
Assessment: 2 days
1. Zoster, Eyelid OS

1. Continue ointment

2. Zoster Iridocyclitis, OS

2. Continue pred forte tid,
atropine instilled in office
Due to increased IOP:
Combigan OS bid

RTO 1 week
Follow up: 1 week later
• Currently on pred forte TID, Combigan BID, OS
only
• Finished Valtrex
• Feels improvement
Examination: 1 week
• VA sc 20/25 OD, 20/40-2
PH 20/25-, OS
• OD unremarkable
• OS
• Lids: healed vesicles, mild
ptosis
• Conjunctiva: Trace injection
• Cornea: Pseudodendrites
with endothelialitis inferiorly
• Anterior Chamber: 2+ WBC

• IOP 17, 20
Assessment: 1 week
1. Redness/discharge of
eye OS

1. Injection improved, less
tearing

2. H. Zoster of eyelid OS

2. Complete resolution

3. Zoster iridocyclitis

3. Durezol QID, atropine in
office (increased IOP);
continue combigan BID
OS

4. H Zoster
keratoconjunctivis

4. Pseudodendrites stable
Follow up 2 weeks later
• Patient reports improvement

• VA sc 20/30 PH 20/25 OD, OS
• OD unremarkable
• OS
•
•
•
•

Lid: no vesicles
Conjunctiva: trace injection
Cornea: punctate staining
Anterior chamber: KP‟s less
evident

• IOP 16, 18 mm Hg
Assessment: 2 weeks
1. H Zoster Iridocyclitis, OS

1. Improvement with quiet
A/C, IOP stable
--Continue Combigan BID OS
--Taper durezol over 2 weeks,
then D/C
--RTO 5 weeks
Follow up 5 weeks later
• Patient currently on
Combigan BID OS only
• VA sc 20/25 OD; 20/30
• OD unremarkable
• OS
• Conjunctiva: tr injection
• Cornea: punctate staining
• Anterior chamber: NO cells

• IOP 16, 18 mm Hg

• Assessment
• H. Zoster Iridicyclitis
• No signs of recurrent
inflammation; IOP well
• D/C combigan
• Follow up 1 month for IOP
check
References
• Cunningham, Emmett. Practical approach to the use of corticosteroids
in patients with uveitis. Can J Opthalmol 2010; 45:352-8
• Moorthy, Ramana. Intraocular Inflammation and Uveitis.American
Academy of Opthalmology. Section 9. pgs 98-116.

• http://www.uveitis.org/uveitis-questionnaire
• http://emedicine.medscape.com/article/1209891-clinical
• “Care of the Patient with Anterior Uveitis,” Optometric Clinical Practice
Guideline. American Optometric Association.

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Uveitis

  • 1. Julie Jantzi, O.D. Mallory Cranmer, O.D. Neal Shastri, O.D. Noushin Ahmed, O.D. Seidenberg Protzko Eye Associates
  • 2. Epidemiology • ~15 per 100,000 in U.S. each year • Causes 10% of blindness in U.S. • Third leading cause of blindness in developed countries • Prevalence varies by location, age, and date of study • Highest among >65 years of age • Females • Chronic and unilateral • Anterior Uveitis most common
  • 3. Pathophysiology • Inflammation of the uvea • Infectious, traumatic, neoplastic, autoimmune, idiopathic • Inflammatory response: chemical mediators result in vasodilation, increased vascular permeability, and chemotaxis of inflammatory cells in eye.
  • 6. Symptoms • Redness • Pain • Photophobia • Epiphora • Blur • Scotoma • Floaters Photo Credit: www.sunglasswarehouse.com
  • 8. Eyelid • Vitiligo • Nodules • Lesions Photo credit: http://www.pcds.org.uk/clinical-guidance/vitiligo
  • 9. Conjunctiva & Episclera • Ciliary Flush • Diffuse injection Photo credit: http://www.mastereyeassociates.com/eyediseases-treatments/iritis---anterior-uveitis/
  • 10. Cornea • Keratic Precipitates • Granulomatous • Non-granulomatous • Fibrin • Pigment • Corneal Edema • Band Keratopathy* Photo Credit: http://www.optometricmanagement.com/articleviewer.aspx?artic leid=71765
  • 11. Anterior Chamber • Cells • Flare (serous) • Pigment • Hypopyon (purulent) • Plasmoid (Fibrinous) • Hyphema + hypopyon (sanguinoid)
  • 13. Iris • Nodules • Busacca • Koeppe • Posterior synechiae • Heterochromia • Granulomas • Atrophy (stromal)* • Cyclitic membrane formation*
  • 14. Angle • Nodule (Berlin) • Vascularization • Peripheral anterior synechiae • Secondary Ciliary body detachment*
  • 15. IOP • Hypotony • Secondary Glaucoma* (Van Bus-kirk EM: Clinical Atlas of Glaucoma. Philadelphia: WB Saunders, 1986.)
  • 16. Vitreous • Cells • Flare • Snowball Opacities • Snowbanking • Active • Inactive • Vitreal Strands Photo credit: Mohawk Valley Retina
  • 17. Retina • Retinal/choroidal inflammatory infiltrates • Sheathing of arteries/veins • Retinal detachment • Schwartz syndrome • RPE hypertrophy/atrophy • Atrophy/swelling of retina, choroid, optic nerve head • Macular edema Photo Credit: Oculist.net
  • 18. Major Complications • Cataracts • Glaucoma • Band Keratopathy • Cystoid Macular Edema
  • 19. Differential Diagnoses • Infections • Non-infectious • Masquerade syndromes • Large cell Lymphoma, retinoblastoma, leukemia, malignant melanoma • Juvenile Xanthogranuloma • Pigment dispersion syndrome • Retinal Detachment • Retinitis pigmentosa • Ocular Ishchemic Syndrome
  • 21. ANTERIOR UVEITIS: POSTERIOR UVEITIS: • • Collagen Vascular Disease • White Dot Syndromes • Acute nongranulomatous Iritis and Iridocyclitis Chronic Anterior Uveitis PANUVEITIS INTRMEDIATE UVEITIS: • • • • Sarcoidosis Sympathetic Ophthalmia • Vogt-Koyanagi-Harada Syndrome • Behcet Disease Pars Planitis Multiple Sclerosis
  • 22. Acute Anterior Uveitis HLA-B27 diseases Glaucomatocyclitic crisis Lens associated uveitiis Postoperative inflammation: IOL associated
  • 23. HLA-B27 DISEASES • Class 1 surface antigen • Seronegative spondyloarthropathies are strongly associated with acute anterior uveitis and HLA-B27 • spondylitis and sacroiliitis Include: • Ankylosing spondylitis • Reactive arthritis syndrome • Inflammatory bowel disease • Psoriatic arthritis
  • 24. Ankylosing Spondylitis • lower back pain and stiffness • men, 20-40‟s • HLA-B27: 90% • dx: sacroiliac imaging studies - “bamboo spine” Ocular findings (40%): • acute anterior uveitis - fibrin and hypopon not uncommon
  • 25. Reactive Arthritis Syndrome • Triad: nonspecific urethritis, polyarthritis, and conjunctivitis often accompanied by iritis • HLA-B27: 95%, young adult men • arthritis in knees, ankles, feet, sacroiliitis in 70% of pts Ocular findings: • Mucopurulent and papillary conjunctivits, acute nongranulomatous iritis in 10% Keratoderma blennorrhagicum
  • 26. Psoriatic Arthritis • Inflammatory arthritis occurs in 30% of pts with chronic psoriasis Inflammatory Bowel Disease • cutaneous changes, distal phalangeal joint inflammation, ungula involvement • • 20% may have sacroiliitis • 25% develop iritis 12% of ulcerative colitis pts and 2.4% of Chrons disease pts develop acute anterior • 20% have sacroiliitis • 60% HLA-B27 positive
  • 27. Glaucomatocyclitic Crisis • Recurrent unilateral mild acute iritis • Elevated IOP, corneal edema, fine KPs, low-grade cell and flare, slightly dilated pupil • Duration: hours to days • Associated with HLA-B54
  • 28. Lens-associated Uveitis • • Immune reaction to lens material: granulomatous or nongranulmatous KPs can be small or large, cells mild to severe, hypopon, PS, elevated IOP, vitritis Postoperative inflammation: IOL-associated Mild inflammation to UGH syndrome (uveitis-glaucoma-hyphema) IOL implantation can activate complement cascade, cellular deposits on IOL, synechiae formation, capsular opacification • Retained lens material • Phthisis bulbi, persistent uveitis, or glaucoma often occurs in these eyes • iris chafing caused by the edges or loops of IOL – mechanical irritation and inflammation
  • 29. Drug Induced Uveitis • Systemic Drugs 1. Rifabutin 2. Bisphosphonates • Topical Drugs 1. Metipranolol 2. Miotics 3. Prostaglandins 3. Sulphonamides 4. Diethylcarbamazine • Intracameral Drugs 1. Cidofovir 5. Cidofovir (intravenous) 2. Antibiotics 3. Urokinase • Vaccines
  • 30. Chronic Anterior Uveitis Juvenile idiopathic arthritis Fuchs heterochromic iridocyclitis Idiopathic iridocyclitis
  • 31. • most common systemic disorder associated with iridocyclitis in children • iritis develops within 5-7 yrs of onset of joint disease • RF: female, pauciarticular onset, ANA • negative RF • eye is usually white and uninflamed, some pts do not have pain • Ocular findings: fine KPS, flare and cells, posterior synechiae, cataract, glaucoma, band keratopathy, vitreous debris, ME, chronic hypotony, phthisis Juvenile Idiopathic Arthritis
  • 32. Fuchs Heterochromic Iridocyclitis • Unilateral uveitis, heterochromia, iris stromal atrophy, small white stellate KPs scattered diffusely over endothelium • synechiae almost never forms but glaucoma and cataracts often occur • Associations with toxoplamosis, HSV, and CMV infections
  • 33. Idiopathic Iridocyclitis • In many cases of chronic iridocyclitis the cause is unknown
  • 35. • absence of infection and systemic disease • 85-90% of intermediate uveitis cases • 5-40 yrs, healthy individuals • associated with HLA-DR15 and HLADR51 alleles Pars Planitis Clinical Characteristics: • 80% bilateral • Ocular manifestations: spillover into anterior chamber, vitreous cells, snowballs, peripheral phlebitis and retinal venous sheathing • Ddx: Syphilis, lyme, sarcoidosis, MS, toxicarasis, lymphoma
  • 36. Multiple Sclerosis • 30% of MS pts will get uveitis • 15% of pts with pars planitis will eventually develop MS • women, 20-50 yrs • HLA-DR2 and DR15 • Granulomatous anterior uveitis, intermediate uveitis, panuveitis
  • 37. Posterior Uveitis Collagen Vascular Diseases Inflammatory Chorioretinopathies of Unknown Etiology
  • 38. Collagen Vascular Disorders Systemic Lupus Erythematosus Polyarteritis Nodosa and Microscopic polyangiitis Wegener‟s Granulomatosis
  • 39. Systemic Lupus Erythematosus • Multisystem autoimmune connective tissue disorder • Type 3 hypersensitivity reaction: Antibody-immune complexes precipitate and cause further immune response • Affects heart, nervous system, joints, skin, lungs, blood vessels, liver, and kidney • Women, 15-45 years • Labs: ANA • Malar “butterfly” rash -70-80%
  • 40. Clinical findings Ocular manifestations in 50% • Keratoconjunctivitis sicca (20%) • Lupus retinopathy -CWS • Cutaneous lesions on the eyelids • Scleral inflammatory disease • Neuro-ophthalmic disorders • Retinal vasculopathy • In rare cases uveitis
  • 41. Polyarteritis nodosa and microscopic polyangiitis • Systemic vasculitis – necrotizing inflammation of medium and small sized arteries • 40-60 yrs, men, hepatitis B • Ocular involvement in 20% • Mortality rate of untreated PAN is 90% by 5 yrs • Labs: p-ANCA, ESR, CRP, tissue biopsy
  • 42. Wegener‟s Granulomatosis • Triad: necrotizing granulomatous vasculitis of upper and lower respiratory tract, glomerulonephritis, necrotizing vasculitis of small arteries and veins • Involvement of paranasal sinuses is most characteristic feature • Ocular involvement in 50%: -- orbital involvement: pseudotumor, cellulitis, dacryocystitis -- anterior, intermediate, or posterior uveitis --scleritis (necrotizing), uveitis, retinal vascular involvement Dx: tissue biopsy, chest x-ray, CRP, ESR, c-ANCA
  • 43. White Dot Syndromes • Group of chorioretinopathies that are inflammatory in nature presenting with discrete, multiple, yellow-white lesions • Symptoms: photopsia, blurred vision, floaters, nyctalopia, enlarged blind spot • Young females
  • 44. • Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) • Birdshot choroidopathy • Multiple evanescent white dot syndrome (MEWDS) • Acute zonal occult outer retinopathy (AZOOR) • Multifocal choroiditis (MCP) • Punctate inner choroidopathy (PIC) • Serpiginous choroiditis
  • 46. Sarcoidoses • Multisystem non-caseating granulomatous disorder of unknown etiology • Intrathoracic : 90%, intraocular: 50% • Affect lymph nodes, skin, eyes, CNS, bones and joints, liver, and heart • African American, females, 20-50 years • Dx: chest x-ray, lymph node biopsy, elevated ACE and lysozyme levels
  • 47. Ocular Findings • can involve any ocular tissue • most common: Anterior granulomatous uveitis –mutton fat KP‟s, koeppe and busacca iris nodules, synechiae • Posterior segment in 20% of ocular sarcoidosis --vitreous infiltration “string of pearls” or “snow balls” --granulomas seen in retinal, choroid --perivascular sheathing, occlusive vascular disease • Eyelid granulomas • Conjunctival nodules • Lacrimal gland infiltration
  • 48. Sympathetic Ophthalmia • Bilateral, diffuse granulomatous, non-necrotizing panuveitis • Autoimmune inflammatory response toward ocular antigens – delayed hypersensitivity to melanin containing structures? • Occurs after surgery or trauma to an eye • 2% of uveitis cases • Anterior Uveitis: mutton fat KPs, PS, thickening of iris from lymphocytic infiltration • Posterior vitritis, dalen-fuchs nodules, peripapillary choroidal lesions, exudative RD
  • 49. • Cell-mediated autoimmune process driven by T lymphocyctes directed against self-antigens associated with melanocytes of all organ systems • Higher association in darkly pigmented ethnic groups • 4% of uveitis referrals in US • Chronic, bilateral, diffuse, granulomatous panuveitis with integumentary, neurologic, and auditory involvement Vogt-KoyanagiHarada Syndrome
  • 50. Stages Acute: bilateral granulomatous anterior uveitis, vitritis, thickening of choroid, edema and hyperemia of optic nerve, multiple serous retinal detachments Convalescent: “sunset glow” fundus, skin changes: vitiligo, alopecia, poliosis Chronic recurrent: repeated bouts of uveitis
  • 51. Behcet Disease • chronic, relapsing, occlusive systemic nongranulomatous vasculitis of unknown etiology • recurrent oral ulcers, skin lesions, genital ulcers, ocular inflammatory disease (70%) • can affect all portions of uveal tract
  • 52. Ocular Findings 80% bilateral • Anterior uveitis: hypopyon in 25% of cases • Posterior segment: necrotizing retinal vasculitis affects both veins and arteries • Optic nerve affected in 25% of pts • Visual prognosis is guarded -25% VA < 20/200
  • 53. Infectious Uveitis •Viral – Herpetic, CMV •Fungal •Protozoal - Toxoplasmosis •Helminthic - Toxocara •Bacterial – Syphilis, Lyme, Tuberculosis, other
  • 54. Viral • Herpes Simplex: • Usually iritis is a keratouveitis but can be: corneal, cutaneous, retinal • arborized • +/- KPs, +/- Hypopyon • HSV-1 is generally oral-labial HSV-2 generally genital infections, although crossover does occur. Image: C. Blake Perry. Source: http://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/HSV-epithelial-keratitis.htm
  • 55. Viral • Herpes Zoster • • • • 72 hours PHN ¼ ocular involvement Infiltrative pseudodendrites (negative stain) Typically elderly and immunocompromised Image:Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 223. Print.
  • 56. • Viral ARN • • • • • • VZV>HSV>CMV Immunocompetent Starts unilateral but 36%Fellow eye Within 6 weeks of onset Painful, Floaters, LOV Peripheral „creamy‟ necrosis that spread, occlusive vascular involvement, Panuveitis • Poor Prognosis, RD in 75% • PORN • VZV mainly • Immunocompromised • Posterior pole involved early • Patchy confluent necrosis, weak or absent uveitis, no vascular involvement • Poor Prognosis, RD in 70% Images: Baumal, Caroline et al. Duane‟s Ophthalmology ,2006.‟Acute Retinal Necrosis and Progresive Outer Retinal Necrosis Syndromes Accessed online: http://www.oculist.net/downaton502/prof/ebook/duanes/pages/v3/v3c028.html#ref
  • 57. Viral • CMV • • • • • • • CD4 <50-100 Most common opportunistic infection in AIDS In congenital cases + birth defects Before HAART ~30% of CD4<50 After HAART incidence decreased 75-80% May start by looking like CWS (small infiltrates) Perivascular hemorrhages, white necrotic retina, frosted branches /vasculitis Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 796. Print.
  • 58. Protozoal • Toxoplasmosis • Toxoplasma gondii • Undercooked meat /cat feces • Most common posterior uveitis in immunocompetent individuals • Often acquired in utero (0.2-1% of pregnancies) • Further Trimester = increased chance of exposure, worst if acquired early (spontaneous abortion) • Majority do not develop ocular manifestations Image: Tabbara. Khalid. „Toxoplasmosis‟. Duane‟s Ophthalmology 2006. Accessed online: http://www.oculist.net/downaton502/prof/ebook/duanes/pages/v4/v4c046.html
  • 59. Protozoal • Toxoplasmosis • „Headlights in the fog‟ • Focal chorioretinal scar with adjacent necrosis and dense vitritis • With AIDS may have multiple lesions • Often self-resolving over months • May use sulfadiazine, pyramethamine, steroids. Image: Tabbara. Khalid. „Toxoplasmosis‟. Duane‟s Ophthalmology 2006. Accessed online: http://www.oculist.net/downaton502/prof/ebook/duanes/pages/v4/v4c046.html
  • 60. Helminthic Parasites • Toxocaraisis • Ingesting eggs of Toxocara • Dogs >cats • Acquired through soil, or uncooked food • Particularly affects kids with pica • Yellow white mass with tractional component, may have vitritis. • Often leads to TRD • Treat uveitis, consider anti-helminthics (albendazole) Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 834. Print.
  • 61. Bacterial • Syphilis • • • • • • Treponema pallidum „Great Mimicker/Masquerader/Imitator‟ Enters through intact mucosa Ocular Syphilis in 2‟ and 3‟ stages (2-6 mo after) Uveitis most common ocular manifestation (up to 10%) Affects immunocompetent or immunocompromised Image: „Syphilis‟. WebMD. Accessed Online: http://www.webmd.com/sexual-conditions/guide/syphilis
  • 62.
  • 63. Bacterial • Syphilis • • • • • • • Dilated iris capillaries (iris roseola) Chorioretinitis, „salt and pepper‟ Vasculitis, vitritis, neuroretinitis, CNV, optic neuritis Granulomatous or non-granulomatous uveitis Argyll Robertson Interstitial Keratitis Tests: • Nontreponemal (VDRL / RPR) • Treponemal (FTA-ABS / MHA- TP) • Tx: Penicillin Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 800. Print.
  • 64. Bacterial • Lyme • • • • • Borrelia burgdorferi 1: rash (e. migrans) 2: dermatologic, neurologic, cardiac 3: Late arthritis, cranial and peripheral nerves 50% reported no hx of tick bite Image: Sources for Rashes: Poster: "Looking for a Bull's-Eye Rash? Look again - erythema migrans can take many forms. A collaborative effort between the Maryland Department of Health and Mental Hygeine and the Lyme Disease Research Foundation of Maryland Maryland.http://www.bayarealyme.org/get-help/lymes-many-symptoms
  • 65. Bacterial • Lyme 1. Conjunctivitis early 2.Motility / CN palsies, optic neuropathy, PTC, chorioretinitis, uveitis 3. Stromal keratitis, symblepharon ELISA, Western Immuoblot Tx: Doxycycline, amoxicillin, if needed ceftriaxone Image: Pediatric Associates of Connecticut. „Tick Bites and Lyme Disease, Accessed Online: http://www.pediatricdoc.com/newsletter.cfm?nid=625
  • 66. Bacterial • Tuberculosis • • • • • • • • • • Mycobacterium tuberculosis Incidence: Annually ~9.3 million patients worldwide Communicable: inhaled aerosolized droplets Primarily involves the lungs Uveitis is common Cough, fever, night sweats Caseating necrotic granulomas CXR PPD / Mantoux testing TB blood tests: interferon-gamma release assays (IGRAs) Images taken from wikipedia . Accessed online: http://en.wikipedia.org/wiki/Tuberculosis_diagnosis
  • 67. Bacterial • Tuberculosis • • • • Granulomatous anterior, intermediate or posterior uveitis Vasculitis with choroiditis, or choroidal tubercles Phlyctenular conjunctivitis Tx: Rifampin, Isoniazid, Pyrazinamide, Ethambutol Image: Yanoff, Myron, and Jay Duker. Ophthalmology. 3rdrd ed. N.p.: Mosby Elsevier, 2009. 808. Print.
  • 68. Infectious Endophthalmitis • Endogenous (<10%) • Systemic via blood stream • Organisms causing endocarditis and GI tract are primary sources, usually with sepsis, immunocompromise, IV drug abuse, catheders. • Typically less pain and inflammation, and can be bilateral • Exogenous • • • • Trauma, Surgery, Infection Acutely is usually gram + (normal flora) Decrease in occurrence over the last 30 years. Bleb-associated may occur at any time. • Acute 0.061-0.3% • Delayed 0.2-18% (variable reports) Image taken from „Endophthalmitis‟ by the American Academy of Ophthalmology 2013. Accessed Online: http://www.aao.org/theeyeshaveit/red-eye/endophthalmitis.cfm
  • 69. Infectious Endophthalmitis • EVS • used <6 weeks, for cataract PO (Acute Onset) • Blur (94%), red eye (82%), pain (74%), hypopyon (86%) • No view of retinal vessels in 79% • Red reflex present in only 32% • Immediate vitrectomy if LP or worse. • B scan • Tx: culture, Vancomycin/Amikacin/Ceftazidime +/Dexamethasone
  • 70. Masquerade Syndromes Accounts for nearly 5% of all patients with uveitis at tertiary referral. Neoplastic Masquerade • Lymphoma • Uveal melanoma • Retinoblastoma • Juvenile xanthrogranuloma • Metastatic tumors Nonneoplastic Masquerade • Retinitis Pigmentosa • Ocular Ischemic Syndrome • Retinal detachment • Intraocular Foreign Bodies • Pigment Dispersion Syndrome
  • 71. Lymphoma • 5th to 7th decade of life • 25% ocular involvement • 15% ocular as initial presentation • 56% with ocular involvement develop CNS involvement • Variable uveitic presentations unresponsive to therapy • Can mimic chronic uveitis
  • 72. Lymphoma: Ocular Findings • Benign reactive uveal lymphoid hyperplasia • Mobile, fleshy episcleral/conj mass • Retinal findings: classic creamy-yellow sub-retinal infiltrates with overlying RPE detachments mimicking sarcoid or birdshot • Anterior uveitis • CME
  • 73. Lymphoma • Diagnosis • MRI • Cerebrospinal fluid analysis • Tissue diagnosis (definitive method) • Treatment • Intravenous and intravitreal methotrexate • Radiation & IV Cytarabine • >60 years = chemo only • <60 years = combination radiation & chemo • Prognosis = poor
  • 74. Uveal Melanoma • 5% ocular inflammation • Episcleritis, ant/post uveitis, endophthalmitis Retinoblastoma • 1-3 % with ocular inflammation • White pseudohypopyon • Conjunctival chemosis • Ultrasound: low internal reflectivity • Diffuse infiltrating variant • 4-6 years old • Limited visibility of fundus
  • 75. Juvenile Xanthogranuloma • Occurs before age 1 • Red-yellow skin lesions • Lesions can involve iris = spontaneous hyphemas • Skin of eyelid involved, globe usually spared • Treatment: topical, periocular, systemic steroids
  • 76. Metastic Tumors • Adults • • Metastic melanoma: brown spherules in retina Most common: Cutaneous Melanoma • Others: white-yellow • Perivascular sheathing, necrotizing retinitis, retinal vasculitis • Lung, GI, breast • Vitritis, serous detachment, CME • Iris nodules, NVI, high IOP • Anterior uveitis • Bilateral and multifocal • Rare
  • 77. Nonneoplastic Masquerade • RP • Waxy disc pallor, attenuation of arterioles, bone-spicule mid-periphery • +FHx • Nyctolopia • Vitritis, CME • ERG depressed early Source: http://www.rpfightingblindness.org.uk
  • 78. Ocular Ischemic Syndrome • Males >65 • CC: decreased vision, mild pain • Corneal edema, A/C reaction, flare>cells, NVI, NVA, • decreased IOP (ischemia), high with NVG • Mild disc edema, dilated tortous veins • mid peripheral hemes • NVD/NVE • FA • Delayed arteriole filling • Diffuse leakage • Capillary non-perfusion • Carotid doppler > 90% diagnostic • Treatment • Endarterectomy • Topical corticosteroids & cycloplegics • PRP for neo • Anti-VEGF injection • 5 year mortality rate of patients 40% • Transient improvement, usually worsens
  • 79. Retinal Detachment • Chronic Peripheral Rhegmatogenous • AC reaction • Vitreous inflammatory & pigment cells • Good vision, may worsen due to CME • Photoreceptor outer segments may be present in AC simulating inflammatory response • IOP elevated, OAG • Schwartz syndrome • DFE with scleral depression • Peripheral demarcation lines, subretinal fluid, retinal breaks, subretinal fibrosis, peripheral cysts http://gridironforgirls.blogspot.com
  • 80. Intraocular FB • Chronic inflammation • Mechanical, chemical, toxic, or inflammatory inflammation of ciliary body • Gonio, ultrasound, CT of eye/orbits • Complications: proliferative vitreoretinopathy and endophthalmitis Pigment Dispersion Syndrome • Pigment released from iris and/or ciliary body • Simulates anterior uveitis
  • 81. Management and Treatment Cycloplegics and mydriatics Corticosteriods Immunomodulating Agents Pressure-lowering medications
  • 82. Cycloplegics Mydriatics • These opthalmic preparations block the responses of the iris sphincter and the accommodative muscle of the ciliary body to cholinergic stimulation, producing pupillary dilation (mydriasis) and paralysis of accommodation (cycloplegia) • Tropicamide 1%, cyclopentolate and atropine have both properties • Phenylephrine has only mydriasis. www.lookfordiagnosis.com/mesh_info.php?term=
  • 83. Cycloplegics Purpose: •To break or prevent posterior synechiae and to relieve ciliary spasm induced photophobia. •Short acting vs. long acting cycloplegics •Dosing depends on the extent of inflammation •(1-2xdaily atropine, 3xdaily tropicamide and phenylephrine)
  • 85. Antigen-dependent lymphocyte activation and STEROID/NSAID activation sites Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with uveitis. Can J Opthalmol 2010; 45:352-8
  • 86. Corticosteroids: Produced through the hypothalamus, pituitary and adrenal axis (HPA) to control inflammation in uveitis http://pubs.niaaa.nih.gov/publications/arcr 344/images/stephens01.png
  • 87. Common uses of corticosteroids -Mainstay of initial therapy Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with uveitis. Can J Opthalmol 2010; 45:352-8
  • 89. Topical Steroids Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with uveitis. Can J Opthalmol 2010; 45:352-8
  • 90. Topical Steroids •Suspension vs. emulsion •Concentration vs. efficacy www.pharmainfo.net/reviews/microemulsionsnovel
  • 91. Potency Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with uveitis. Can J Opthalmol 2010; 45:352-8
  • 95. Intravitreal Injections Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with uveitis. Can J Opthalmol 2010; 45:352-8
  • 97. Systemic NSAIDS • Complications of prolonged use: -myocardial infarction -hypertension -stroke (selective Cox-2 inhibitors) www.mayoclinic.com/health/medic www.ohioinjurylaw.com/ohioattorney/medical-mal
  • 98. Immunomodulating Medications (IMT) • Antimetabolites • T cell signaling Inhibitors • Alkylating Agents • Biologic response modifiers www.riskindoc.com/new_psoria
  • 99.
  • 100. Indications for IMT  inadequate response of uveitis to corticosteroid therapy for greater than 3 months with doses exceeding 5-10mg/day.  contraindications to corticosteroid use: 1. (diabetes, hypertension, peptic ulcer, GERD, immunocompromised state, psychiatric conditions) 2. exacerbated side effects 3.chronic corticosteroid dependence www.fortiusfitness.com/blog/steroi d-side-effects
  • 101. Precautions  No infection present  No hepatic or hematologic contraindications  Close physician follow up  Teratogenic mamaprayed.blogspot.com/2012/08/if-you-give-m
  • 102. Antimetabolites • Azathioprine, methotrexate, mycophenolate mofetil • Azathioprine (purine nucleoside analogue) -interferes with DNA replication and RNA transcription -found beneficial in VKHsyndrome, int. uveitis, Behcet uveitis, sympathetic opthalmia and necrotizing scleritis -GI side effects (nausea, upset stomach) are cause for D/C. -CBC, LFT every 4-6 weeks. -Thiopurine S-methyltransferase (TPMT) testing (metabolizes 6mercaptopurine,ie 6-MP) www.lookfordiagnosis.com/mesh_info.php?term
  • 103. Methotrexate • Folic Acid analogue • Inhibitor of dihydrofolate reductase and it’s DNA replication • Causes extracellular accumulation of adenosine to create an anti-inflammatory effect. • Useful for JIA (first line choice IMT treatment for children), sarcoidosis, panuveiti s, various other types of uveitis • Prospective study for IV inj. for refractory uveitis and uveitic CME. www.humira.com/jia/what-is-jia.aspx
  • 104. Mycophenolate Mofetil • Inhibits inosine monophosphate dehydrogenase and DNA replication • Side effects: reversible GI distress, diarrhea; 20%pt. pop. • CBC monthly • 85% effectivity in chronic uveitis patients and a good second choice IMT for children.
  • 105. T Cell Signaling Inhibitors Cyclosporine and tacrolimus: • Calcineurin inhibitors- disrupt T-cell receptor signal transduction and down regulate IL-2 transcription and CD4 T lymphocyte receptor expression. Sirolimus: • Non-calcineurin inhibitordisrupts T cell signaling that inhibits Ab production and Blymphocytes. www.bdbiosciences.com/research/tcell/reg
  • 106. Cyclosporine • SE: Nephrotoxicity and systemic hypertension; paresthesia,hypertrichosis. • BP, CBC, serum creatinine monthly. • Used for int. uveitis and posterior uveitis cases from Behcets and VKH. • Used in combination with corticosteroids for modest improvement
  • 107. Tacrolimus • AE: nephrotoxicity • Low dose and increased potency to cyclosporine • Less risk of hypertension and hyperlipidemia • Useful for chronic int. and posterior uveitis.
  • 108. Sirolimus • One open-label, prospective study it was found useful in treatment for refractory noninfectious uveitis • GI side effects • Under active investigation for use in treating uveitis
  • 109. Alkylating Agents Cyclophosphamide • Active metabolites alkylate purines in DNA and RNA, creating impaired DNA replication and cell death • Cytotoxic to dividing and resting lymphocytes • AE:myelosuppression, hemorrhagic cystitis, sterility Chlorambucil • Interferes with DNA replication • AE:myelosuppression, sterility Treatment for int. uveitis, VKH, SO, Behcet Significant AE: increased risk for malignancy (leukemia, various cancers)
  • 110. Infliximab Biologic Response Modifiers • Chimeric, monoclonal IgG1k antibody against TNFalpha • Treatment for Behcet, sarcoidosis and VKH. Some HLA B27 ant. uveitis • Drug induced toxicity (lupus, systemic vascular thrombosis, CHF, malignancy, demyelinating disease and vitreous hemorrhage) • +PPD, contraindication • Adalimumab, less AE, same effectiveness
  • 111. Alternative Therapy Under FDA Investigation • Rituximab:chimeric monoclonal antibody against CD20 positive cells (B lymphocytes) • Daclizumab: monoclonal Ab to IL-2 • IFNalpha2a: antiviral, immunomodulatory and antiangiogenic effects (leukopenia and thrombocytopenia side effects)
  • 112. Management/Follow up • Encounter visit determines extent of ocular inflammation (ant., int., panuveitis, etc). • Make a differential list and send out for further pertinent lab work to confirm/rule out differentials. super-trainer.com/fitness-business-becoming-a-bu
  • 113. Anterior Uveitis • Cycloplegic • Steroid • Dosage depends on severity • Follow up in 2-3 days • If 2-3 additional follow up appt. prove no improvement, referral to uveitis specialist medlibes.com/entry/anterior-uveitis
  • 114. Intermediate/Posterior Uveitis • Determine extent of inflammation, if anterior involvement, start on cycloplegic and steroid. • Refer to retinal specialist for periocular steroid injections (if deemed non-infectious) • Intravitreal injections for chronic anterior uveitis or inflammation not responsive to periocular injection www.retinaeye.com/uveitis.html
  • 115. Uveitic Treatment Adverse Effects • Steroid responders: Need to lower IOP by utilizing beta blockers, alpha2 agonists,CAI, prostaglandins and their combinations where appropriate.
  • 116. Case #1 • 70+ yr old cauc. male • Redness, mild pain, blurry vision in the right eye. • Slit lamp: 2+ AC WBC reaction; florrid NVI,NVA, corneal edema with descemet folds; IOP 16mmHg OD
  • 118. OIS! • DFE: mid-peripheral blot hemorrhages • No CME, NVD, NVE • Tx: Prednisolone Forte q2hrs, Atropine BID OD • Referral to retinal consult for PRP and possible injections of anti-vegf • Contact PCP for carotid doppler, echocardiogram, EKG
  • 119. Case #2 • 17 yr cauc. female • CC: redness, pain, photophobia, bl urry vision • Review of systems was unremarkable • Second episode, first episode two years ago in GA.
  • 120. Review of Systems • General - Weight loss, fatigue, fever Skin - Rash, nodules, changes in nails • Neck - Lymphadenopathy • Respiratory - Cough, wheezing • Cardiac - Chest pain/discomfort, dyspnea • Gastrointestinal - Hepatomegaly, diarrhea, frequent bowel movements • Genitourinary - Delayed secondary sexual characteristics • Musculoskeletal - Muscle or joint pain, arthritis, back pain, limitation of motion (refer to uveitis.org for more information and questionnaire)
  • 121. Differentials? • JIA, lyme disease, lupus, sarcoid • ????? • Ran pertinent lab tests for her age (CBC c diff.,ANA, ACE, Lyme titer,ESR,RF, HLA B27) • Durezol q2hrs, Atropine BID OD. Pledget of 10% phenylephrine to break posterior synechiae • CBC with diff., lyme titre, RF, ANA, ACE, serum lysozyme • Blood results…negative. Consult with uveitis specialist.
  • 122. Case 3
  • 123. Chief Complaint • 46 year old WM • Redness OS x 5 days, gradually getting worse that is constant throughout the day • (+) photophobia, epiphora • ( - ) eye pain, flashes, floaters, cold sores • He was seen at Patient First 3 days ago where they diagnosed him with shingles over the left eyelid, noting ocular involvement • Treated with 20 mg Pred, mild pred taper and 1 gm of Valtrex tid x 10 days
  • 124. History • Medical History • Hypertension, GERD • Treated with Omeprazole and Diovan • Family Medical History • Unremarkable
  • 125. Examination • VA sc 20/25 OD, OS, OU • OD unremarkable findings • OS • Lid: vesicles along upper forehead & nose, lid erythema, small hordeolum • Conj: nasal injection
  • 126. Examination • Cornea • Punctate Epithelial Keratitis • Inferior stromal haze • Anterior Chamber • Cells – WBC 2+ • ( - ) Flare • IOP 16, 19 mm Hg • Fundus • Unremarkable OU
  • 128. Assessment 1. Redness/Discharge of Eye OS 1. Educated patient on findings 2. H. Zoster Keratoconjunctivitis OS 2. Neomycin ung on vesicles, BID 3. H. Zoster Iridocyclitis, OS 3. Pred Forte TID, OS 4. H. Zoster of Eyelid 4. Cool compresses and antibiotic ung on vesicles
  • 129. Follow Up: 2 days later • Patient now complains of eye pain in the morning • Thinks it is getting worse • Currently taking pred forte tid OS and will finish Valtrex the following week
  • 130. Examination: 2 days • VA sc 20/25 OD; 20/40 OS PHNI • OD unremarkable • OS Lid: Vesicles along upper forehead & nose with lid erythema, hordeolum on superior lid • Cornea • Prominent pseudodendrites • Anterior Chamber • 2+ WBC • IOP 16, 27 • Fundus: Unremarkable OU
  • 131. Assessment: 2 days 1. Zoster, Eyelid OS 1. Continue ointment 2. Zoster Iridocyclitis, OS 2. Continue pred forte tid, atropine instilled in office Due to increased IOP: Combigan OS bid RTO 1 week
  • 132. Follow up: 1 week later • Currently on pred forte TID, Combigan BID, OS only • Finished Valtrex • Feels improvement
  • 133. Examination: 1 week • VA sc 20/25 OD, 20/40-2 PH 20/25-, OS • OD unremarkable • OS • Lids: healed vesicles, mild ptosis • Conjunctiva: Trace injection • Cornea: Pseudodendrites with endothelialitis inferiorly • Anterior Chamber: 2+ WBC • IOP 17, 20
  • 134. Assessment: 1 week 1. Redness/discharge of eye OS 1. Injection improved, less tearing 2. H. Zoster of eyelid OS 2. Complete resolution 3. Zoster iridocyclitis 3. Durezol QID, atropine in office (increased IOP); continue combigan BID OS 4. H Zoster keratoconjunctivis 4. Pseudodendrites stable
  • 135. Follow up 2 weeks later • Patient reports improvement • VA sc 20/30 PH 20/25 OD, OS • OD unremarkable • OS • • • • Lid: no vesicles Conjunctiva: trace injection Cornea: punctate staining Anterior chamber: KP‟s less evident • IOP 16, 18 mm Hg
  • 136. Assessment: 2 weeks 1. H Zoster Iridocyclitis, OS 1. Improvement with quiet A/C, IOP stable --Continue Combigan BID OS --Taper durezol over 2 weeks, then D/C --RTO 5 weeks
  • 137. Follow up 5 weeks later • Patient currently on Combigan BID OS only • VA sc 20/25 OD; 20/30 • OD unremarkable • OS • Conjunctiva: tr injection • Cornea: punctate staining • Anterior chamber: NO cells • IOP 16, 18 mm Hg • Assessment • H. Zoster Iridicyclitis • No signs of recurrent inflammation; IOP well • D/C combigan • Follow up 1 month for IOP check
  • 138.
  • 139. References • Cunningham, Emmett. Practical approach to the use of corticosteroids in patients with uveitis. Can J Opthalmol 2010; 45:352-8 • Moorthy, Ramana. Intraocular Inflammation and Uveitis.American Academy of Opthalmology. Section 9. pgs 98-116. • http://www.uveitis.org/uveitis-questionnaire • http://emedicine.medscape.com/article/1209891-clinical • “Care of the Patient with Anterior Uveitis,” Optometric Clinical Practice Guideline. American Optometric Association.

Hinweis der Redaktion

  1. To help manage and treat uveitis, especially bilateral, recurrent, or recalcitrant uveitis, it is imperative that the underlying cause is found. Here is a brief overview on different disease and conditions that have been associated with uveitis.
  2. Most common form of uveitis
  3. Negative RFClass 1 surface antigen that presents to T suppressor cellsDisease are sometimes clinically indistinguishable5% of population, about ½ of pts with acute iritis
  4. --improves with exertion, bamboo spine is conplete fusion of the spineis a chronic inflammatory disease of the axial skeleton with variable involvement of peripheral joints and nonarticular structuresNot all HLA-B27 develop the disease, only 1 in 4 will develop spondyloarthritis or eye diseaseShould be referred to rheumotologist, NSAIDs are mainstay of tx of AS, anti-TNF are gaining popularity-physical therapy and smoking cessation can slow down disease progression
  5. triggered by an infection in another part of your body — most often your intestines, genitals or urinary tract.--in addition to classic triad they can have scaly, erythematous disorder of the palms and soles of feetConjunctivitis most common, uveitis -10% can become bilateral and chronic b/c of permanent breakdown of blood-aqueous barrier
  6. IBD: may also develop slerouveitis, these pts are usually HLAB27 –ve compared to those with acute iritisPA: dx based on typicl cutaneous changes -erythematous hyperkeratotic rash
  7. Symptoms are vague: discomfort,blurred vision, halos--recurrences are very common over many years--rare, dx of exclusion, other syndromes must be r/o such as herpetic uveitis
  8. can occur following disruption of the lens capsule (traumatic or surgical) or leakage of lens protein in hypermature cataracts--other names include phacoanaphylacticendopthalmitis, phacotoxic uveitis and phacolytic glaucoma--unsure of mechanism but thought due to immune rxn to lens protein--now with modern IOL’s less complications
  9. relatively rare occurrence and is reported to represent less than 0.5 % of cases in a tertiary referral uveitis clinic (1). The time between medication use and occurrence of symptoms varies, ranging from a few days to months.. The cause for drug-induced inflammation is not well known. Postulates include direct and indirect mechanisms. Direct mechanisms are typically seen soon after medication use, typically occurring with topical or intracamerally instilled drugs. Indirect mechanisms include immune complex deposition in uveal tissues, immune reactions to antigens released from antibiotic-induced death of microorganisms, or an alteration of melanin’s ability to scavenge free radicals (2).
  10. Inflammation of the anterior segment persistent and relapses less than 3 months after d/c of therapy
  11. --pauciarticular onset 85=90% of JIA that have uveitis: 4 or fewer joints are involved in 1st 6 months or disease, may have no joint symptoms--because of freq. asymptomatic nature of uveitis in these pts profound silent ocular damage can occur, long term prognosis often depends on extent of damage at time of dx--pts should be evaluated by rheumatologist and undergo ANA testing--joint pain can be minimal or absent at time of uveitis diagnosesDdx: fuchs, sarcoid, behcet, seronegativespondy., herpetic and lyme disease
  12. Usually overlooked, 3% of cases referred to uveitis clinics are dx w fuchs--usually vague symptomsdx: made due to distribution of KP’s, lack of synechiae, lack of symptoms, and heterochromiafew require therapy, topical steroids can lessen inflammation but do not resolve itLighter colored eye indicated involved, although in blue eyed the darker b/c stromal atrophty and darker iris pig epith shows
  13. 15% of all cases of uveitis’--inflammation in ant vitreous and the vitreous base overlying ciliary body and peripheral retina-pars plana complex--may aggregate in the vitreous “snow balls”, accumulate on inf pars plana –”snowbanking”
  14. In older main complaint is floatersComplications: cataract (60%), glauc, CME(5)%), retinal neovas. (15%), VH, tractional or rhegmatogenous RD-- CME develops and becomes chronic and refractory in 10% of pts--ischemia from phlebitis with angiogenic stimuli from intraocular inflammation can lead to neovascularization 10% of casesthought to be autoimmune reaction against vitreous, peripheral retinal and CB
  15. Can preceed dx of MS in 25% of pts and by 5-10yrs15% of pts with pars planitis may eventually develop MSME less common then pars planitis, also less mild interm. uveitis
  16. SUN: Inflammation involving the retina and or choroid. Inflammatory cells may be observed diffusely throughout the vitreous cavity overlying foci of active inflammation
  17. B lymphocyte hyperactivity, polyconal B-lymphocyte activation, hypergammaglobulinemia, autoantibody formation, and T-lymphocyte autoreactivity with immune complex deposition
  18. KCS most common symptomLupus retinopathy is most well recognized post. Segment manifestation and is considered and important marker of systemic disease -3% to 29% in severe –w or w/o hemesVascular occlusiveLupus choroidopathy- choroidal infarction, CNV, serous elevations of RPE and retina
  19. Consequence of Underlying vascular disease: Post pole: may manifest as HTN retinopathy, retinal arteriolar occlusive disease, choroidal infarcts, choroidaliscehmia, neuro-ophthmanifestions, scleritis, optic atrophy, peripheral ulcerative keratitis
  20. 15% ocularinvolv. At presentation, 50% during course of disease
  21. Level of retinal, RPE , choriocapillaris and choriodDdx: syphilis, DUSN (diffuse unilateral subacuteneuroretinitis, OHS, as well as non infectios: sarcoidosis, sympathetic ophthalmia, VKH, intraocular lymphoma
  22. Many of these have viral or autoimmune component--Serpiginouschoroiditis is associated with HLA B-27
  23. Involvement of all anatomical compartments of the eye –AC, vitreous, retina and choroidUsually bilateral
  24. abnormal collections of chronic inflammatory cellsgranulomas) that can form as nodules in multiple organs.[1] The granulomas that appear are usually not of the caseating variety and are most often located in the lungs or the lymph nodes, but virtually any organ can be affected. uveitis being most frequent manifestation
  25. Cutaneous invovment –orbital and eyelid granuloma’s, conj. Nodules, lacrimal gland infiltration -KCS2/3 pts with ocular sarcoidosis have ant. Uveitis-
  26. Usually occurs 3 months after injury (80%) to 1 year (90%)--characteristic white, midequatorialchoridal lesions (Dalen nodules) that may become confluent, peripapillarychoridal lesionslymphocytic infiltration and the formation of granulomas. Deposition of these granulomas between Bruch&apos;s membrane and the RPE is termed a Dalen-Fuchs&apos; nodule--injured eye “exciting eye” and noninjured is “sympathizing” eye--dx: clinical, ddx VKH
  27. The prodromal stage is marked by flu-like symptoms. Patients present with headache, nauseadysacusia and tinnitus.
  28. Sunset glow –resolution of exudative detachments,depigmentation of the choroid
  29. Oral ulcers are the most frequent finding BD –recurrent mucosal ulcers the produce sign discomfort and pain (round white ulcerations with red rims, occur every 5-10 days or every month)
  30. AO and VO’s, combination, vascular sheathing with variable amounts of vitirtis –becomes very ischemic--can look like ARN due to ischemic nature of vasculitis and accompanying retinitis.--can have papillitis, ischemic optic neuropathiesHLA associations: HLAB12, HLA B27, HLA B51IMT is essential due to chronic inflamm.
  31. Based on maryland 2011 data from the CDC:Maryland ranked 3rd in the US in reports of primary and secondary syphilisMD reported 452 cases of primary and seconday syphilis in 201124 cases of congenital syphilis in 2011.
  32. Short acting agents like tropicamide are useful for acute non-granulomatous anterior uveitis. Chronic uveitis and moderate flare anterior chamber reactions for disease like juvenile idiopathic arthritis (JIA) require chronic use of short acting agents to prevent further complications like synechiae. If there is a significant uveitic reaction like granulomatous inflammation, the use of long acting agents like atropine are useful to prevent the accumulation of inflammatory cells by stabilizing the ciliary body vascular system.Long acting agents like atropine are useful for
  33. Corticosteroids have both direct and indirect inhibitory effects on the potent pro-inflammatory transcription factor NF-kB.
  34. Periocular injections (either transseptal or sub-tenon approach) of triamcinolone acetonide or methylprednisolone should not be used in infectious uveitis cases such as toxoplasmosis. Also these injections can cause high IOP spikes with increased duration.
  35. Suspension needs to be shaken, emulsion does not.
  36. Higher concentration does not mean higher potency, only high corneal permeability or penetration into the anterior chamber,can yield higher potencies
  37. COX1 and 2 inhibitors-commonly used in ocular inflammationCOX 2 only inhibitors (celecoxib,rofecoxib,valdecoxib)-increased risk of adverse cardiovascular events, therefore these are not used for ocular inflammation or are limited in scope-Systemic NSAIDS: useful for chronic iridocyclitis (ie. JIA-associated iridocyclitis) and potentially for CME to allow maintenance on a lower dose of topical NSAIDS.Topical NSAIDS: limited role in ocular inflammation, not used in the treatment of anterior uveitis, but only implicated in postoperative pseudophakic CME and episcleritis.
  38. These medications take 2-4months for successful control of ocular inflammation. Therefore, it is necessary to start these medications while the corticosteroids are being tapered to not further delay of successful treatment.
  39. Corticosteroid-induced side effects:pars planitis (int. uveitis), retinal vasculitis, panuveitis, and chronic iridocyclitis.
  40. Low/no TPMT activitiy: do not use aziothioprineInt.:reduced dosage &lt;50mg/dayNormal/High: higher doses
  41. Can create dissemination of tuberculosis