Core Ophthalmic Knowledge For Uveitis

Core Ophthalmic Knowledge for Uveitis
Symposia Chair: Ramana S Moorthy MD

Course: SYM10
Saturday, October 12, 2019
2:00 PM - 3:45 PM
Room: SOUTH 205
Published: 10-11-19 7:01 PM
Join the conversation: #aao2019

® 2019 American Academy of Ophthalmology. All rights reserved.
Core Ophthalmic Knowledge
Uveitis Review
AAO 2019
Saturday, October 12th
MOC Exam Review Course Faculty
• Ramana S. Moorthy, M.D.
o Former Uveitis POC Panel Chair
o Clinical Associate Professor, Indiana University School of Medicine, Indianapolis, IN
• Russell W. Read, M.D., Ph.D.

o Uveitis POC Panel Chair
o Professor, University of Alabama at Birmingham, AL
• Wendy M. Smith, M.D.

o Uveitis POC Panel Member
o Assistant Professor of Ophthalmology, Mayo Clinic, Rochester, MN
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Financial Disclosures
• Dr. Moorthy has no financial conflicts of interest to disclose.
• Dr. Read has no financial conflicts of interest to disclose.
• Dr. Smith has no financial conflicts of interest to disclose.
• The following Academy staff have no financial conflicts of interest to disclose:
Beth Wilson, Rayna Ungersma and Tony Ching.
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Overview of Curriculum
• Classification of Uveitis and Clinical Approach
• Non-infectious Uveitis
• Infectious Uveitis
• Ocular Manifestations of Acquired Immune Deficiency Syndrome (AIDS)
• Masquerade Syndromes
• Medical Therapy
• Important Core Uveitis Concepts
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Classification of Uveitis
• Anatomic
• Onset and course (acute vs. chronic)
• Granulomatous vs. non-granulomatous
o Clinical rather than histologic descriptor
• Infectious vs. non-infectious
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• Anatomic – Anterior Uveitis
o Primary site of inflammation is anterior chamber
▪ Iritis
▪ Iridocyclitis
• Anatomic – Intermediate Uveitis

o Primary site of inflammation is vitreous
o Includes pars planitis (idiopathic)
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• Anatomic – Posterior Uveitis
o Primary site of inflammation is retina or choroid
▪ Choroiditis
▪ Chorioretinitis
▪ Retinochoroiditis
▪ Retinitis
▪ Neuroretinitis
• Anatomic – Panuveitis
o No predominant site of inflammation
o Inflammation in anterior chamber, vitreous and retina and/or choroid
o Do not consider structural complications such as CME or neovascularization
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• Timing
o Onset of episode
▪ Sudden
▪ Insidious
o Course of episode
▪ Limited – less than 3 months
▪ Persistent – greater than 3 months
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• Course of Disease - Characterization
o Acute
▪ Attacks are of sudden onset and limited duration
o Recurrent
▪ Repeated episodes activity separated by period of inactivity, off treatment, of > 3 months
o Chronic
▪ Persistent uveitis
▪ Relapse with d/c therapy
The Standardization of Uveitis Nomenclature (SUN) Working Group. Standardization of uveitis nomenclature for reporting clinical data.
Results of the First International Workshop. Am J Ophthalmol 2005;3:509-516.
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A 25-year-old patient presents with subacute onset of
blurred vision and light sensitivity in his right eye.
Examination reveals ciliary flush, anterior chamber
cell and flare, and keratic precipitates without
posterior signs of inflammation. What is the working
diagnosis?
1. Infectious retinitis
2. Pars planitis
3. Anterior uveitis
4. Posterior scleritis
C-UVE001
11
• Anterior Uveitis Symptoms • Anterior Uveitis Signs
o Acute o Acute
▪ Sudden onset ▪ Limbal injection
▪ Pain, redness, photophobia ▪ KP
▪ Definite start date ▪ AC reaction (+/- hypopyon)
▪ Self-limited ▪ Posterior synechiae, PAS
o Chronic ▪ IOP often low
▪ Insidious onset o Chronic
▪ May be asymptomatic ▪ Usually no injection
▪ Floaters, decreased vision ▪ KP
▪ History often less precise ▪ AC reaction
▪ Long duration ▪ Posterior synechiae, PAS
▪ IOP often high
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Anterior Uveitis
Photographs courtesy of Debra A. Goldstein, MD & Ramana S. Moorthy MD.

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• Anterior Uveitis Signs - • Anterior Uveitis Signs -
Nongranulomatous Granulomatous
o Small KP o Mutton fat KP (may leave “ghost
o May have Koeppe nodules (small) KP”)
o No Busacca nodules o Large Koeppe nodules
o DDx includes HLA-B27, sarcoidosis, o Busacca nodules
Herpes, Fuchs’ iridocyclitis o Choroidal granulomata
o DDx includes sarcoidosis, TB, VKH,
herpes, toxoplasmosis
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Anterior Uveitis: Non-granulomatous Anterior Uveitis: Granulomatous
Photographs courtesy of Debra A. Goldstein, MD

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Anterior Uveitis: Non-granulomatous Anterior Uveitis: Granulomatous

Severe acute iridocyclitis in HLA-B27 positive patient Mutton fat KP

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What is a clinical finding of intermediate uveitis?
1. Subfoveal choroidal neovascularization
2. Exudative retinal detachment
3. Exudates over the pars plana
4. Punched-out peripheral chorioretinal scars
C-UVE002
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• Intermediate Uveitis Symptoms • Intermediate Uveitis Signs
o Floaters o Vitreous cells
o Decreased vision o Vitreous strands
o Usually insidious onset o Snowball opacities
o Exudates over pars plana (snow
banking)
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Intermediate Uveitis
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• Posterior Uveitis Symptoms • Posterior Uveitis Signs
o Blurred vision o Retinal or choroidal infiltrates
o Floaters o Optic nerve inflammation
o Flashes o Inflammatory sheathing of retinal
o Metamorphopsia vessels
o Asymptomatic
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Posterior Uveitis
Photographs courtesy of Ramana S. Moorthy, MD
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Posterior Uveitis
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• Panuveitis - Signs • Endophthalmitis - Symptoms & Signs
o Anterior, intermediate and posterior o Pain and visual loss (can be profound)
segments of uveal tract involved with o Diffuse conjunctival injection
no predominant site of inflammation
o Lid edema sometimes with ptosis of the
(cells)
upper lid
o Combinations of signs from anterior,
o Hypopyon often with fibrin
intermediate, and posterior uveitis
o Vitritis – often dense
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Clinical Approach to Uveitis
• Comprehensive History
o Onset of this episode o Past medical history
▪ Sudden ▪ Medication history – particularly for drug
induced uveitis
▪ Insidious
▪ History of autoimmune disorders
o Laterality
▪ Prior ocular surgery
▪ Unilateral
o Complete review of systems
▪ Bilateral
o Family history
o Pattern including previous episodes
▪ Chronic o Social history: smoking, travel, occupation,
drug use
▪ Recurrent acute
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Clinical Approach to Uveitis: Examination
• Examination • Slit lamp exam
o Best corrected visual acuity o Conjunctival nodules/granulomas
o Pupillary function o Cornea
o Motility, confrontation fields ▪ Epithelium-dendrites
▪ Stroma- Infiltrates or cells
▪ Endothelium
 KPs:
- Size: small, non-granulomatous; large,
granulomatous
- Color: white, pigmented
- Distribution: Arlt’s triangle, diffuse
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• Slit Lamp Exam
o Anterior Chamber
▪ Depth – Shallow - ? angle closure
▪ Cells - ? hypopyon, hyphema, pigment cells
▪ Flare - ? fibrinous
▪ Structural changes (peripheral anterior synechiae,
rubeosis)
o Iris:
▪ Color, contour, ? heterochromia, atrophy
▪ Posterior or anterior synechiae
▪ Nodules (Koeppe, Busacca)
o Lens: ? cataract
Photograph courtesy of H. Nida Sen, MD, MHSc
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• IOP
o Applanation tonometry
• Perform gonioscopy in patients with

elevated IOP or hypotony
• Fundus
o Macula
▪ CME?
▪ Atrophy?
o Vessels, retina, choroid, optic nerve
o Examine retinal periphery
Photograph courtesy of Wendy M. Smith, MD
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• Testing
o Specific tests for syphilis (e.g., Syphilis IgG)
o Sarcoidosis: CXR, ACE, consider CT chest
o TB: CXR, PPD or interferon gamma release assay (QuantiFERON - TB Gold)
o Other tests based on clinical features (tailored approach)
▪ e.g., HLA-B27 for acute anterior uveitis
▪ e.g., ANA for chronic iritis in child
28
Non infectious ocular inflammation
• Acute anterior uveitis
• Persistent (chronic) iridocyclitis
• Juvenile idiopathic arthritis
• Sympathetic ophthalmia
• Scleritis
29
For a patient with acute iridocyclitis with
hypopyon, what serologic test will most likely be
positive?
1. Human leukocyte antigen (HLA) B27
2. Antinuclear antibody (ANA)
3. Fluorescent treponemal antibody (FTAbs)
4. HLA-B51
C-UVE003
30
Acute Anterior Uveitis
• History
o Sudden onset o Postoperative iridocyclitis
o Pain, redness, photophobia ▪ Acute postoperative
 Redness, pain, photophobia
o Vision may be decreased
 May be due to too rapid taper of topical
o Patterns corticosteroids
▪ One episode or recurrent ▪ Delayed postoperative iridocyclitis
▪ Unilateral or bilateral  Can have episodic redness, pain, and
▪ Same eye or alternating photophobia due to exacerbations
 Usually becomes persistent and unresponsive to
topical corticosteroids
 Infectious cause – most commonly
Propionibacterium acnes
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• Features
o Ciliary flush
o Non-granulomatous or granulomatous KP
o AC flare and cells
o With severe disease, hypopyon may be present
o Often have retrolenticular/anterior vitreous cells (iridocyclitis)
o Posterior synechiae
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• Differential Diagnosis
o HLA-B27 – with or without systemic disease (e.g., ankylosing spondylitis, reactive arthritis)
o Sarcoidosis
o Viral: herpes simplex, herpes zoster
o Syphilis
o Endophthalmitis
o Trauma
o IOFB
o Tubulointerstitial nephritis and uveitis syndrome
o Idiopathic
33
(© 2006 Ramana S Moorthy MD. Used by permission.)

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• Medical therapy
o Topical corticosteroids
o Cycloplegia (help with photophobia, break/prevent posterior synechiae)
o Therapy for severe or recurrent attacks
▪ Periocular corticosteroid injection (once infection ruled out in patients who do not develop steroid
response)
▪ Oral corticosteroids
▪ Immunomodulatory agent
o Consider infection in all patients with first episode of severe uveitis
35
• Disease Related Complications
o Chronic posterior synechiae & pupillary seclusion
o PAS
o Elevated IOP/glaucoma
o Macular edema
o Cataract
36
Patients with what form of juvenile idiopathic
arthritis are most likely to demonstrate chronic
iridocyclitis as a feature?
1. Oligoarticular
2. Juvenile ankylosing spondyloarthropathy
3. Systemic (Still's disease)
4. Polyarticular
C-UVE004
37
Chronic iridocyclitis is typically characterized by
which of the following ?
1. Pain
2. Photophobia
3. Insidious onset
4. Redness
C-UVE005
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Persistent (Chronic) Iridocyclitis
• Etiology
o Often idiopathic
o Associated with systemic disease
▪ Juvenile idiopathic (rheumatoid) arthritis
▪ Sarcoidosis
• History
o Onset is usually insidious, but can have acute onset
o Blurred vision
o Floaters
o May be asymptomatic
o Pain, redness and photophobia are rare, although may occur at onset and intermittently
throughout course
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• Features
o Keratic precipitates
o AC cell
o AC flare, may be significant, even
with few cells
o Anterior vitreous cells
o Iris nodules
o Posterior synechiae
Photograph courtesy of Debra A. Goldstein, MD.
40

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• Medical therapy
o Topical corticosteroid
o Mydriatic/cycloplegic (to prevent formation of posterior synechiae)
o Local corticosteroid injection
o Systemic therapy
▪ Oral corticosteroids
▪ Immunomodulatory therapy
 Referral to uveitis specialist or rheumatologist
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• Complications
o Posterior synechiae are frequent
o PAS
o Ocular hypertension/glaucoma – check IOP at every visit
o Hypotony
o Iris atrophy
o Macular edema – frequent cause of decreased vision
o Cataract, both because of underlying inflammatory disease and corticosteroid use
o Band keratopathy: much more common in children
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• Patient Instructions
o Nature of chronic disease (i.e., we cannot cure the disease, and patient may not be able
to be tapered off of drops)
o Necessity of using drops even in absence of symptoms
o Requirement for follow-up, even in absence of symptoms
▪ For example, may develop asymptomatic glaucoma secondary to corticosteroid drops
44
Juvenile Idiopathic Arthritis Uveitis
• Etiology
o Associated with oligo-articular subtypes of JIA
o Most common with positive ANA
• History
o Most have history of joint disease
o Asymptomatic, painless loss of vision
o More common in females
o More common with early onset arthritis (age 2 – 5)
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• Features
o Bilateral non granulomatous iridocyclitis
o Chronic disease, not recurrent acute
o Majority develop after onset of arthritis
o Activity does not parallel activity of joint disease
o Highest risk within 2 years of onset of arthritis, most within 7 yrs
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Photograph courtesy of Debra A. Goldstein, MD. 47


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• Management
o Mild disease
▪ Topical corticosteroids
▪ Mydriatics
o More severe disease
▪ Oral Prednisone – short courses only
▪ Steroid sparing agents
o Rheumatology consultation for management of immunomodulatory therapy and joint
disease
o Early referral to uveitis specialist if inflammation is not controlled
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• Complications of the disease
o Calcific band keratopathy
o Posterior and anterior synechiae
o Cataract; Glaucoma – check IOP, examine nerve
o Hypotony; Amblyopia; Blindness
o The disease is painless and children may not exhibit any symptoms
▪ Regular follow-up for active disease is required
▪ Regular screening per published guidelines for those at highest risk (oligoarticular disease ,
ANA-positive, girls) is required
50
Following repair for corneal laceration with uveal
prolapse in the right eye, a 34-year-old woman had
surgical closure with residual light-perception vision.
Two months later, she develops light sensitivity and
optic disc edema in the left eye. What is the most
likely diagnosis?
1. Temporal arteritis
2. Sympathetic ophthalmia
3. Harada's disease
4. Behçet's disease
C-UVE006
51
Sympathetic Ophthalmia
• Epidemiology
o Uncommon,
o Bilateral, granulomatous diffuse panuveitis
o Following penetrating injury to one eye (exciting eye)
o Time to onset is variable: 2 weeks to many years following penetrating ocular
trauma/surgery
▪ Latent period followed by development of uveitis in both the exciting and uninjured globe
(sympathizing eye)
▪ Most cases occur in first three months
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• History
o Bilateral photophobia, redness, blurring of vision usually 10 days or more after ocular
injury
• Features
o Bilateral panuveitis
o KP, often granulomatous
o Vitritis
o Multifocal, yellow-white choroidal lesions
o Peripapillary choroidal lesions
o Exudative retinal detachment
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o Bilateral uveitis following penetrating ocular injury or surgery should suggest sympathetic
ophthalmia
o Vogt-Koyanagi-Harada syndrome
o Sarcoidosis
o Tuberculosis
o Syphilis
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(© 2006 Russell W. Read, MD. Used by permission.)

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Photograph courtesy of Albert T. Vitale, MD

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Photograph courtesy of Wendy M. Smith, MD

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• Patient Management
o Early enucleation within first few weeks of trauma may prevent SO
o Once SO is established, enucleation likely not beneficial
▪ Do not enucleate eye with potential for vision
o With advances in treatment, the exciting eye may turn out to be the eye with the better
visual acuity
o Treatment
▪ Topical corticosteroids and cycloplegic agents for anterior chamber inflammation
▪ Systemic corticosteroids for posterior segment inflammation
▪ Immunomodulation as extended therapy is anticipated in most patients
58
For 4 days, a 60-year-old woman has had a
painful red eye and photophobia. There is a
violaceous appearance to the scleral vessels.
Her anterior chamber is quiet. What is her most
likely diagnosis?
1. Bacterial conjunctivitis
2. Herpes zoster ophthalmicus
3. Anterior uveitis
4. Scleritis
C-UVE007
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Scleritis
• Symptoms and Signs
o Severe pain radiating to the jaw or temple (can interfere with sleep because of severity
▪ Pain is characteristic feature of scleritis and not episcleritis
o Red violaceous hue due to deep scleral injection
▪ Redness does not blanche with topical Neo synephrine like episcleritis or conjunctivitis
o Edema to the episcleral and scleral tissues
• Etiology
o 50 % - Idiopathic
o 40% - Systemic connective tissue, vasculitis
o 10% - Infectious diseases
60
Scleritis
• History
o Most commonly present in fourth to sixth decade
o Insidious onset of moderate to severe deep ocular pain
▪ Frequent radiation to jaw, forehead, sinuses
o Redness
▪ Sectoral (interpalpebral) or diffuse
o Photophobia, epiphora
o Exquisite tenderness to palpation
▪ Pain absent in scleromalacia perforans
61
Scleritis
• Features
o Tortuous, congested vessels of the deep episcleral plexus
o Violaceous appearance to sclera (bluish –red color)
▪ Blanches incompletely with topical 10% phenylephrine
o Nodules (non-mobile)
o Avascular zones with sequestra, uveal show in cases of necrotizing scleritis
o Spontaneous perforation rare
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Scleritis
Associated with Systemic Autoimmune Diseases

(© 2006 Ramana S Moorthy MD. Used by permission.) 63
Scleritis
Associated with Systemic Autoimmune Diseases

64
Scleritis
• Risk Factors
o Systemic inflammatory diseases
▪ Rheumatoid arthritis – most common
▪ Granulomatosis with polyangiitis (formerly known as Wegener's)
• Patient Management
o Oral Non-steroidal anti-inflammatory agents (NSAIDs)– mild cases
o Oral Corticosteroids
o Immunomodulatory therapy – for severe and necrotizing scleritis
▪ Refer to uveitis specialist and rheumatologist
65
Scleritis
• Disease Complications
o Spontaneous perforations rare in necrotizing scleritis
o Glaucoma – due to elevated episcleral venous pressure
66
Infectious Uveitis
• Herpes Zoster (Varicella-Zoster Virus) Iritis
• Necrotizing Herpetic Retinitis: Acute Retinal Necrosis
• Toxoplasmic Retinochoroiditis
67
Varicella zoster iridocyclitis can often be
distinguished from non-herpetic anterior uveitis
by what clinical characteristic?
1. Posterior synechiae
2. Cranial nerve V2 dermatitis
3. Young age
4. Ocular hypertension
C-UVE008
68
The most important sign suggesting high risk
for ocular involvement from herpes zoster
infection of the first division of the trigeminal
nerve is:
1. Forehead numbness
2. Periocular pain
3. Hutchinson’s sign
4. Lid marginal lesions
C-UVE009
69
Herpes Zoster (Varicella-Zoster Virus) Iritis
• Etiology
o Associated with herpes zoster ophthalmicus (HZO) (VZV involving the first branch of
trigeminal nerve)
• History
o Usually have a history of ipsilateral herpes zoster skin lesions on forehead
o May have lesion on tip of nose (Hutchinson sign)
o Pain, redness, photophobia are common
• Features
o Corneal disease
o IOP may be elevated
o KPs
70
Photograph courtesy of Debra Goldstein 71

• Risk factors
o Relative immunosuppression
▪ Advanced age
▪ Secondary to immunosuppressive agent
▪ Leukemia, lymphoma, HIV infection
• Differential diagnosis
o HSV iritis
• Medical therapy
o Systemic antiviral therapy: Ideally started within 72 hours of skin lesions
o Topical corticosteroids
o Cycloplegia
72
• Diagnosis
o Serology for antiviral IgG and IgM of no benefit unless completely negative
o Aqueous Paracentesis
▪ PCR of aqueous sample
▪ Can be evaluated for HSV1 and 2, VZV, and CMV
• Complications related to therapy
o Acyclovir is well tolerated in patients
with normal renal function
▪ Neutropenia
▪ Monitor renal function annually
▪ Hallucinations, seizures
• Disease related complications

o Glaucoma
o Iris atrophy
o Mydriatic pupil
Photograph courtesy of Dr. Debra Goldstein
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A patient with a history of herpes zoster virus presents with
blurred vision, eye pain, and floaters in one eye. This eye is
found to have peripheral retinal necrosis with discrete
borders. What treatment should be instituted to prevent
involvement in the fellow eye?
1. Topical ganciclovir
2. Systemic steroids
3. Intravitreal steroids
4. Systemic acyclovir
C-UVE010
75
Necrotizing Herpetic Retinitis: Acute Retinal
Necrosis
• Etiology
o Varicella zoster virus
▪ Most common agent
o Herpes simplex virus
o Cytomegalovirus
▪ Least common
• Symptoms
o Decreased vision
o Floaters
o Pain may occur in patients with acute retinal necrosis
76
Necrosis
• Signs
o Retinal vasculitis: arteritis o In immunocompetent hosts – usually acute
retinal necrosis
o Peripheral retinal necrosis and
whitening with discrete borders ▪ More inflammation in vitreous since host can
mount an immune response
o Rapid progression in the absence of
▪ Less rapid progression of retinitis
antiviral therapy
o In immunocompromised hosts (e.g. AIDS) –
o Begins in periphery, spreads
Progressive Outer Retinal Necrosis (PORN)
centrally
▪ Less inflammation in vitreous (especially if
o Cells and flare in anterior chamber low CD4 counts)
and cells in vitreous, with or without
▪ More rapid progression
optic disc swelling or hyperemia
77
Necrosis
Photograph courtesy of Ramana S. Moorthy, MD

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Necrosis
Photograph courtesy of Ramana S. Moorthy, MD

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Necrosis

Necrotizing Herpetic Retinitis: Progerssive
Outer Retinal Necrosis (in AIDS)

81
Necrosis
• Management
o Prompt referral to uveitis or retina specialist
▪ Diagnosis
 Aqueous Paracentesis
- PCR of aqueous sample
- Can be evaluated for HSV1 and 2, VZV, and CMV
o Antivirals
▪ Systemic
▪ Intraocular
o Corticosteroids
▪ Systemic- NEVER ALONE
 Only with appropriate anti-viral coverage
82
Necrosis
• Complications
o Prognosis is better if
▪ Peripheral disease that is caught early and treated aggressively
o Prognosis is poor if
▪ Large areas of retinitis, especially macular
▪ Early optic nerve involvement
▪ Structural complications – retinal detachment
▪ Monotherapy with steroids
o Long term complications
▪ Retinal detachment risk is high (>50%)
▪ Involvement of fellow eye, antivirals to reduce risk
Photograph courtesy of Ramana S. Moorthy, MD 83

Toxoplasmic Retinochoroiditis
• Etiology – Toxoplasma gondii
o Congenital vs acquired infection
o Reactivation vs newly acquired
• History
o Maternal infection → congenital infection
o Acquired disease much more common than previously appreciated, exposure to areas
where cats are located
• Symptoms
o Unilateral floaters or blurred vision
o May have symptoms related to AC reaction: Pain, redness, photophobia
84
• Features
o Newly acquired disease: may be no scar
o Recurrent disease: unilateral focal retinitis adjacent to healed chorioretinal scar
o Atypical forms of extensive retinochoroiditis can occur in immunocompromised individuals
▪ AIDS
▪ Over age 70 years
o Active retinitis is yellow-white and slightly elevated
o Intraocular inflammation
▪ Iritis
▪ Vitritis
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o Infections
▪ Necrotizing herpetic retinitis
▪ Syphilis
▪ Toxocariasis
o Masquerade syndromes
▪ Intraocular lymphoma
o Autoimmune
▪ Behçet’s
86
• Diagnosis - Clinical
o Serologies : Anti-Toxoplasma IgG and IgM – confirmatory
o Vitrectomy and vitreous PCR for Toxoplasma gondii
• Management
o Treatment dependent upon location of lesion
o Medical treatment options include a variety of anti-protozoal agents
o Oral corticosteroids may be used in combination with anti-toxoplasmic therapy but should
not be used alone
o Do not use steroids alone without antimicrobial coverage
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Congenital Toxoplasmosis
88
Recurrent Toxoplasmosis
89

91
92

Photograph courtesy of Albert T. Vitale, MD
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Acquired Immune Deficiency Syndrome
Related Disease
• Ocular Manifestations of AIDS
95
Cytomegalovirus retinitis most commonly
occurs in HIV-positive patients who have which
of the following?
1. CD4 < 50 cells/mm3
2. Detectable or high HIV viral loads
3. CMV detectable in urine
4. Elevated serum anti-CMV IgG and IgM titers
C-UVE011
96
An asymptomatic HIV-positive patient is sent for an
eye exam. The CD4+ cell count is 150/L. There are
scattered hemorrhages and a few cotton-wool spots.
What is the most likely diagnosis?
1. Progressive outer retinal
necrosis (PORN)
2. Cytomegalovirus retinitis
3. Pneumocystis jiroveci (formerly
Pneumocystis carinii) (PCP) dissemination
4. HIV retinopathy
C-UVE012
Image with permission from Cunningham ET Jr, Belfort R Jr. <HIV/AIDS and the Eye: A Global
Perspective. Ophthalmology Monograph 15. San Francisco: American Academy of Ophthalmology; 2002:55 97
Ocular Manifestations of AIDS
• HIV Retinopathy
o Common and usually asymptomatic
o Cotton wool spots, retinal hemorrhages and microaneurysms
98
• CMV Retinitis
o Still most common ocular opportunistic infection in AIDS
o Most common with CD4 count less than 50
o Hemorrhagic retinal necrosis
o Perivascular involvement
o CMV retinitis associated with a substantial risk of vision loss despite treatment
▪ Causes of vision loss
 Retinal detachment
 Retinitis involving optic nerve or macula
 Cystoid macular edema
99
CMV Retinitis
100
CMV Retinitis
Photograph courtesy of Debra A. Goldstein
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CMV Retinitis
Photograph courtesy of Debra A. Goldstein 102
• CMV Retinitis Differential Diagnosis
o Acute retinal necrosis (ARN)
o Progressive outer retinal necrosis
o Toxoplasmic retinochoroiditis
o Syphilitic Chorioretinitis
103
Acute Retinal Necrosis

Photograph courtesy of Debra A. Goldstein 104
Progressive Outer Retinal Necrosis in AIDS patient

Photograph courtesy of Ramana S. Moorthy
105
• CMV Retinitis – Treatment
o Inevitably progressive without treatment
▪ Anti-CMV therapy
▪ Highly active anti-retroviral therapy (HAART)
o Ganciclovir
▪ Intravenous
▪ Intravitreal injection
▪ Intravitreal implant (no longer available)
o Foscarnet
▪ Intravenous
▪ Intravitreal injection
o Valganciclovir: PO
108
• Herpes Zoster Ophthalmicus -
Features
o Painful cutaneous vesicular eruption
along CN V1 (ophthalmic division)
o Redness
o Decreased vision
o Corneal epithelial changes
o AC cells and flare
o Vitreous cells
o Optic neuritis
o At risk for necrotizing retinitis
(© 2006 Debra A Goldstein MD. Used by permission.)
109
• Herpes Zoster Ophthalmicus – Patient Instructions
o Refer patient to a specialist immediately
▪ Infectious disease specialist for highly active anti-retroviral therapy (HAART) and other systemic
therapy
▪ Retina or uveitis specialist for ocular manifestations
o Early treatment may ameliorate late consequence
110
A previously healthy 69-year-old woman complains of
recent-onset bilateral decreased vision and floaters. Her
daughter states that she has also had a significant decline
in memory. During the retinal examination, the
ophthalmologist should be on the alert for what clinical
feature?
1. Multiple retinal tears
2. Bone spicule-like pigmentation
3. Multifocal subretinal infiltrates
4. Bilateral posterior vitreous detachments
C-UVE013
111
A 70-year-old man presents with confusion, weakness, and
decreased vision. His fundus exam reveals clumps and
sheets of white cells in the vitreous and multifocal subretinal
and subpigment epithelial infiltrates in the affected eye.
What diagnostic or therapeutic step would you
recommended?
1. Vitreous biopsy
2. B-scan ultrasound
3. Systemic steroids
4. Intravitreal steroids
C-UVE014
112
Masquerade Syndromes
• Neoplastic masquerade syndrome
o Primary central nervous system intraocular lymphoma
113
Neoplastic Masquerade Syndromes
• Epidemiology
o Lymphoma arising in the eye or associated with the primary central nervous system
lymphoma (PCNSL)
▪ Primary intraocular lymphoma is usually a diffuse large B cell lymphoma
o Older patients - 6th and 7th decade
o Incidence increasing
114
• History
o Central nervous system (CNS) signs and symptoms
▪ Confusion, weakness, deterioration in mental functions
o Ocular history
▪ Bilateral, asymmetric
▪ Decreased vision and floaters
▪ Eye can be first site of presentation of PCNSL
115
• Clinical Features
o Clumps and sheets of white cells in the vitreous
o Multifocal subretinal and sub-pigment epithelial infiltrates
• Testing and Evaluation

o Referral to uveitis or retinal specialist
▪ Work-up including vitreous biopsy
116
PCNSL
117
o Sarcoidosis
o Syphilis
o Tuberculosis
o Necrotizing herpetic retinitis
o Toxoplasmosis
o Acute posterior multifocal placoid pigment epitheliopathy
• Risk Factors
o Age
o Immunosuppression
o AIDS
118
• Management
o Corticosteroids
▪ Cytolytic to lymphoma cells, leads to apparent response to treatment initially
▪ Decrease the yield of vitreous biopsy
o Immediate referral to an oncologist for treatment and staging
o Median survival rate of PCNSL is 3-6 months with supportive care alone
▪ With treatment, longest median survival approaches 40 months
• Complications
o Death
o Visual impairment or blindness
119
Which topical agent provides the longest
duration of cycloplegia?
1. Atropine 1%
2. Scopolamine 0.25%
3. Cyclopentolate 2%
4. Tropicamide 1%
C-UVE015
120
Which systemic complication is most likely to
occur with the use of oral nonsteroidal anti-
inflammatory drugs (NSAIDs)?
1. Pancytopenia
2. Peptic ulcer
3. Deep venous thrombosis
4. Hemorrhagic cystitis
C-UVE016
121
The use of topical NSAID would be expected to
be ineffective in treating:
1. Episcleritis
2. Scleritis
3. Non-infectious keratitis
4. Inflammation following cataract surgery
C-UVE017
122
Topical steroids may increase the frequency of
what ocular problem?
1. Subconjunctival hemorrhage
2. Hypotony
3. Vitreous hemorrhage
4. Retinal detachment
C-UVE018
123
Patients on chronic long-term systemic
corticosteroids should have which of the
following test annually?
1. Total body bone density scan
2. Liver function tests
3. Serum cortisol levels
4. Creatinine clearance
C-UVE019
124
Medical Therapy of Uveitis
• Cycloplegic drops
• Non-Steroidal Anti-Inflammatory Agents (NSAIDs)
• Corticosteroids
o Topical
o Periocular
o Intravitreal
o Systemic
125
Cycloplegics
• Indications
o AC reaction
▪ To prevent/reduce formation of posterior synechiae
▪ To decrease photophobia and pain due to ciliary and sphincter muscle spasm
▪ To break recently formed posterior synechiae
• Contraindications
o Allergy or sensitivity to agent or to others in its class
o Occludable angles
126
Cycloplegics
• Pre-procedure/Therapy Evaluation
o Question for history of allergy or adverse reaction
o Check angle depth because of possibility of inducing angle-closure glaucoma
• Agents - listed in decreasing order of duration of effect

o Atropine 1%
o Scopolamine 0.25%
o Homatropine 5% and 2% (no longer available?)
o Cyclopentolate 2%, 1% and 0.5%
127
Cycloplegics
• Complications
o Tachycardia o Psychosis, acute psychotic reaction
o Fever ▪ Limit dosage to no more than
recommended frequency
o Urinary retention
▪ Most case reports are in the pediatric
o Blurred vision age group
▪ Can be minimized by use of a short ▪ Treat with supportive care
acting cycloplegic
o Allergic reaction
▪ Temporary use of reading glasses
128
Non-Steroidal Anti-Inflammatory Agents
(NSAID)
• Indications
o Noninfectious ocular inflammatory disease, especially scleritis (oral NSAID use) or
episcleritis (oral or topical)
o May be useful as adjunct during tapering of topical corticosteroids
o Analgesia
o For oral use
▪ Renal insufficiency or other kidney disease
▪ Peptic ulcer disease
▪ Bleeding diathesis
o Allergy or insensitivity to agent or others in its class
130
(NSAID)
• Complications
o Oral
▪ Renal insufficiency
▪ Gastritis/peptic ulcer
▪ Nausea
▪ Decreased clotting ability
▪ Abnormal liver enzyme
o Topical
▪ Corneal epithelial breakdown, thinning, erosion, ulceration
▪ Ocular wound healing delay
▪ Ocular bleeding
▪ Conjunctival hyperemia
131
(NSAID)
o Inform the physician of any new symptoms while on the medication
▪ Bleeding
▪ Increased bruising
▪ Changes in stool
▪ Changes in urination
o Take with food
o Do not use aspirin containing products concurrently
132
Corticosteroids
• Indications
o Inflammatory disease
o May be used cautiously in infections as adjunct to antimicrobial agent
o Infectious etiology, unless covered by appropriate antimicrobial agents, e.g., Toxoplasma
retinochoroiditis
o Poorly controlled or difficult to control diabetes (for systemic, but not topical
corticosteroids)
o History of psychosis
o Necrotizing scleritis (for topical, periocular)
133
Corticosteroids
• Alternatives
o Periocular, intraocular, topical corticosteroids (for isolated anterior disease)
o Immunosuppressive agents
▪ Anti-metabolites – methotrexate, azathioprine, mycophenolate
▪ Inhibitors of T-cell signaling – Cyclosporine, tacrolimus
▪ Alkylating agents – cyclophosphamide, chlorambucil
▪ Biologic response modifiers – infliximab, adalimumab, tocilizumab, rituximab
134
Corticosteroids
• Administration
o Oral
o Intravenous
o Intramuscular (rarely used for eye disease)
o Sub-Tenon
o Intraocular
▪ Intravitreal - dexamethasone
▪ Injection of suspension - preservative free triamcinolone
▪ Polymer matrix - dexamethasone
▪ Sutured implant – fluocinolone acetonide
o Topical
135
Corticosteroids
• Complications – All Forms of Tx
o Posterior subcapsular cataracts (not reversible)
o Increased intraocular pressure (often reversible)
• Complications – Systemic Use

o Fluid and electrolyte disturbances: Sodium retention, fluid retention, CHF in susceptible
patients, potassium loss, hypertension
o Musculoskeletal: Muscle weakness, vertebral compression fractures, aseptic necrosis of
femoral and humeral heads, pathologic fracture of long bones
o Gastrointestinal: Peptic ulcer, erosive gastritis
o Dermatologic: Impaired wound healing, thin fragile skin, petechiae, ecchymoses
136
Corticosteroids
• Complications – Systemic Use Continued…
o Neurological: Increased ICP, convulsions
o Endocrine: Menstrual irregularities; development of Cushingoid state; suppression of
growth of children; manifestations of latent diabetes mellitus; increased requirements for
insulin or oral hypoglycemic agents in persons with diabetes
o Ophthalmic: Posterior subcapsular cataract, increased IOP
o Additional reactions: Urticaria and other allergic, anaphylactic or hypersensitivity reactions.
Weight gain, fat redistribution, sleep disturbance, infection, reduced symptoms from
infection, adrenal suppression, accelerated atherosclerosis
137
Corticosteroids
• Complication – Orbital Use
o Unintended injection into choroidal or retinal circulation
o Perforation of the globe
▪ Retinal detachment
▪ Vitreous hemorrhage
o Ptosis (more common with superior injections)
o Proptosis
o Orbital fat atrophy, fibrosis
o Orbital fat prolapse (with inferior retroseptal injections)
o Subconjunctival hemorrhage, chemosis
o Pain from injection
138
Corticosteroids
• Complications – Intravitreal Use
o Intraocular inflammation
▪ Sterile, toxic-type reaction (with preserved formulations)
▪ Infectious endophthalmitis
o Vitreous hemorrhage
o Retinal detachment
• Complications – Topical Use

o Worsening of external infectious disease
o Increased incidence and frequency of spontaneous subconjunctival hemorrhages
139
Corticosteroids
• Follow-Up Care • Patient Instructions
o Systemic use o Inform the physician of any new symptoms
▪ Long-term use should be minimized while on the medication
 Particularly doses >10mg/day of o Avoid exposure to infection
prednisone
o Maintain physical activity and optimal body
o Topical (regional, intravitreal) weight
▪ Monitor IOP and cataract status o Persons with diabetes should monitor
blood glucose frequently and adjust
treatment accordingly, in conjunction with
primary care physician
140
Case 1: 55 year old white male presents with
gradual, painless vision loss in the left eye. His
wife thinks that his left eye is changing color
• Vision
• OD 20/20
• OS 20/400
• IOP
• OD 21
• OS 34
• Exam
• OD Mild NS, otherwise normal DFE
• OS
141
Case 1
142
Case 1
143
Case 1: As a comprehensive ophthalmologist,
what next step would be most beneficial for the
patient?
1. Begin predniolone drops QID and cycloplegics QHS in left eye
2. Refer immediately to a uveitis specialist
3. Examine the left eye for iris heterochromia
4. Proceed with a laboratory work up : FTA, Quantiferon TB, Chest X-ray, ACE
C-UVE020
144
Case 2: A 50 year old healthy white female
presents with progressive vision loss of 2 -3
weeks duration in the left eye
• PMHx - NC
• Meds - None
• Vision
o OS 20/400
o OD 20/20
• Exam
o OD normal
o OS
▪ SLE: F/C = 2/2, granulomatous KPs
▪ Vitreous 3+ cells
▪ Fundus
145
Case 2
• Laboratory testing: FTA – negative

• PPD skin test: 0 mm induration
• ACE and Lysozyme: Normal
• Chest Xray: Normal
146
Case 2: What is most likely diagnosis?
1. Necrotizing herpetic retinitis
2. Retinal vasculitis from Behçet disease
3. Syphilitic chorioretinitis
4. Tuberculous retinal vasculitis
C-UVE021
147
Case 2: As a comprehensive
ophthalmologist what should you do next to
improve the visual outcome for the patient?
1. Initiate immediate treatment with systemic corticosteroids
2. Begin posteior subtenon’s corticosteroid injections
3. Perform Ozurdex injection
4. Refer immediately to a retina/uveitis specialist
C-UVE022
148
Case 3: 67 year old white male with 6 month
history of progressive vision loss in each eye.
• PMHx - Negative (never hospitalized)
• Meds - None
• Exam
o VA
▪ OD HM
▪ OS 20/100
o A/C - No cells
o Vitreous - 3+ cells in sheets both eyes
o Fundus
149
Case 3
150
Case 3
151
Case 3: What would be the next step in the
work up of this patient?
1. Lab testing: FTA, Quantiferon TB
2. Aqueous PCR for VZV, HSV, and CMV
3. Vitreous and retinochoroidal biopsy
4. Lab testing is not indicated
C-UVE023
152
Case 3: What is the diagnosis?
1. Toxoplasmosis
2. Primary CNS/Intracular Lymphoma
3. Non-infectious uveitis
4. CMV retinitis
C-UVE024
153
Case 3
154
Case 3
155
Case 3
156
Case 3
• Diagnosis: PCNSL
• Management
o Systemic Chemo Rx – IV MTX, Ara-C
o Radiation therapy – low dose fractionated
o Intravitreal therapy – MTX 600mcg + Rituximab 1mg x3 for 3 months
• Patient still alive at 3 years with NLP right eye and 20/70 vision in left eye
• Prognosis: Much poorer with CNS symptoms
157
Case 4: A 75 year old man on home oxygen
presents to you with complaints of redness,
pain, and photophobia in his left eye
• PMHx
o COPD
o Recently MAI lung infection
o HIV negative
• Vision
o OD 20/20
o OS 20/60
• Exam
o OD normal
o OS
158
Case 4
• Vitreous – No cells
• Retina – Normal
159
Case 4: Which of the following laboratory tests
must be considered in the work-up of this
patient?
1. HLA B27
2. RPR
3. ANA
4. Rheumatoid factor
C-UVE025
160
Case 4
• Lab testing
o FTA – Neg
o ACE/Lysozyme – Normal
o CXR – Chronic parenchymal scarring in upper lobes
o PPD – 0mm
o HLA-B27 – neg
161
Case 4: Which of the following is an appropriate
next step to secure the diagnosis?
1. Review current systemic medications
2. Aqueous paracentesis
3. Immediate Vitrectomy
4. Repeat HLA-B27 testing
C-UVE026
162
Case 5: 30 year old Asian male presents
with a 2 week history of sudden painless vision
loss in the left eye
• PMHx • Ta: 12 OU
o 30 lb weight loss in 6 months
• SLE
o Fevers, swollen “glands”
o OD Normal
• Meds - None o OS Dendritic KPs, F/C - 1/1
• VA • DFE
o OD 20/20
• OD Normal
o OS HM
• OS
163
Case 5
164
Case 5
165
Case 5: What is the most likely diagnosis?
1. Toxoplasmosis
2. Syphilis
3. CMV retinitis
4. HIV retinopathy
C-UVE027
166
Case 5: What is the most important serologic
testing that is needed?
1. FTA-ABS
2. Anti-HSV IgG and IgM antibody titers
3. CMV urine culture
4. HIV serology
C-UVE028
167

Core Ophthalmic Knowledge For Uveitis

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Core Ophthalmic Knowledge For Uveitis

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Core Ophthalmic Knowledge for Uveitis

Symposia Chair: Ramana S Moorthy MD

Join the conversation: #aao2019

• Russell W. Read, M.D., Ph.D.

• Wendy M. Smith, M.D.

• Infectious vs. non-infectious

• Anatomic – Intermediate Uveitis

Photographs courtesy of Debra A. Goldstein, MD & Ramana S. Moorthy MD.

Anterior Uveitis: Non-granulomatous Anterior Uveitis: Granulomatous

Photographs courtesy of Debra A. Goldstein, MD

Anterior Uveitis: Non-granulomatous Anterior Uveitis: Granulomatous

Photographs courtesy of Debra A. Goldstein, MD

• Perform gonioscopy in patients with

(© 2006 Ramana S Moorthy MD. Used by permission.)

Photograph courtesy of Debra A. Goldstein, MD.

Photograph courtesy of Debra A. Goldstein, MD.

Photograph courtesy of Debra A. Goldstein, MD. 47

Photograph courtesy of Debra A. Goldstein, MD.

(© 2006 Russell W. Read, MD. Used by permission.)

Photograph courtesy of Albert T. Vitale, MD

Photograph courtesy of Wendy M. Smith, MD

Associated with Systemic Autoimmune Diseases

Associated with Systemic Autoimmune Diseases

Photograph courtesy of Debra Goldstein 71

• Disease related complications

Photograph courtesy of Dr. Debra Goldstein

Photograph courtesy of Ramana S. Moorthy, MD

Photograph courtesy of Ramana S. Moorthy, MD

(© 2006 Ramana S Moorthy MD. Used by permission.) 80

(© 2006 Ramana S Moorthy MD. Used by permission.)

Photograph courtesy of Ramana S. Moorthy, MD 83

(© 2006 Ramana S Moorthy MD. Used by permission.) 90

(© 2006 Ramana S Moorthy MD. Used by permission.)

Photograph courtesy of Albert T. Vitale, MD

Acute Retinal Necrosis

Progressive Outer Retinal Necrosis in AIDS patient

• Testing and Evaluation

• Agents - listed in decreasing order of duration of effect

• Complications – Systemic Use

• Complications – Topical Use

• Laboratory testing: FTA – negative

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