Uveitis Flashcards
(193 cards)
1
Q
What is bilateral diffuse uveal melanocytic proliferation?
A
• Rare paraneoplastic
• Melanocytic proliferation of choroidal melanocytes
2
Q
How do you treat blebitis?
A
• Topical antibiotics only
3
Q
What are the signs of bleb-associated endophthalmitis and what is the treatment?
A
• Eye pain
• Poor vision
• Hypopyon
• Vitritis
• Treatment: Intravitreal antibiotics with or without
PPV
4
Q
Allopurinol increases the toxicity of which immunomodulatory medication?
A
• Azathioprine
• Mechanism: allopurinol inhibits xanthine oxidase
which inactivates 6-mercaptopurine (active form of
azathioprine). This leads to a build up of
6-mercaptopurine resulting in increased effect and
toxicity
5
Q
Treating which blood disorder with chlorambucil results in a 13.5-times risk of developing leukemia?
A
• Polycythemia rubra vera
6
Q
What is the mechanism of action and side effect profile of chlorambucil?
A
• Alkylating immunomodulatory therapy that
interferes with DNA replication
• Side effects: 13.5-fold increased risk of leukemia
when used for polycythemia rubra vera,
myelosuppression, infertility
7
Q
What is the mechanism of action and side effect profile of cyclophosphamide?
A
• Alkylating agent
• Side effects: 33-fold increased risk of bladder
cancer, hemorrhagic cystitis, infertility
8
Q
What are the imaging and lab findings that help support the diagnosis of congenital lymphocytic choriomeningitis (LCM)?
A
• Brain imaging which classically shows
periventricular intracerebral calcifications (As
opposed to more diffuse calcifications in congenital
toxoplasmosis)
• Positive serologic testing for LCM virus IgM and IgG
9
Q
Under what CD4 count is a patient at risk for CMV retinitis?
A
• CD4 count < 50 cells/mm3
10
Q
What is the mechanism of action and side effect profile of rituximab?
A
• Monoclonal IgG antibody directed against CD20
antigen on the surface of human B lymphocytes
• Side effects: depletion of B cells, reduction of IgG
and IgM levels for 6-12 months following therapy
11
Q
Which test may give false positive results for patients with ocular leptospirosis?
A
• Leptospirosis is a gram negative spirochete
• Therefore, it can cause falsely positive rapid
plasma reagin (RPR) or FTA-Abs
12
Q
Which types of posterior uveitis do NOT present with vitritis?
A
• Presumed ocular histoplasmosis syndrome
• Punctate inner choroiditis
• Progressive Outer Retinal Necrosis
• Serpiginous choroidopathy
• Subacute Sclerosing Panencephalitis
13
Q
What is the mechanism, route of administration, indication, and side effect profile of cidofovir?
A
• Cytidine nucleoside analogue that causes inhibition
of DNA synthesis
• IV administration
• Used for induction and maintenance therapy for
CMV retinitis
• Longer half life
• Side effects: renal damage, anterior uveitis,
hypotony
14
Q
Which oral antibiotics have the best vitreous penetration?
A
• Fluoroquinolones
15
Q
What cells are targeted initially by HIV? How does HIV affect these cells?
A
● CD4 T cells
○ HIV decreases the number of CD4 cells
● Macrophages
○ HIV alters the function of macrophages
16
Q
When is treatment of toxoplasmosis always indicated?
A
• Congenital Toxoplasmosis
• Pregnant women with acquired disease
• Immunocompromised patients (HIV/AIDS,
neoplastic disease or immunomodulating therapy)
17
Q
What is leukotriene B4 and what is its effect?
A
• Inflammatory mediator that causes lysosomal
enzyme release and oxygen radical formation
18
Q
What is the classic presentation, exam findings, and fluorescein angiogram (FA) pattern of punctate inner choroidopathy (PIC)?
A
Presentation
• Typically a young, myopic, healthy woman with
bilateral changes
Exam findings
• Small 100-200 micron focal lesions confined to posterior
pole that can progress to atrophic/pigmentary
chorioretinal scars; minimal vitreous reaction
FA pattern
• Early hypofluorescence of inflammatory lesion with late
staining; early hyperfluorescence can also occur
especially if CNV is present
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19
Q
What is the classic presentation, exam findings, and fluorescein angiogram (FA) pattern of multifocal choroiditis (MCP)?
A
Presentation
• Young, myopic woman with bilateral involvement
Exam findings
• Diffuse retinal lesions between 50-200 um with vitritis
• Peripheral chorioretinal streaks and peripapillary
atrophy, similar to ocular histoplasmosis syndrome
• Lesions are typically larger and more pigmented than
those seen in PIC
FA pattern
• Early hypofluorescence with late staining of active
lesions
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20
Q
What is the classic presentation, examination, and IVFA findings of Acute Zonal Occult Outer Retinopathy (AZOOR)?
A
• Patient is classically a young, myopic woman with
photopsias and progressive visual field loss that
begins as enlarged blind spot; unilateral 60% of
time at presentation
• Exam shows early lesions as multiple white-gray
dots with normal RPE. Later exam shows RPE
atrophy and hyperpigmentation resembling bony
spicules of RP
• Early IVFA findings may be normal, showing only
prolonged retinal circulation time; late IVFA
findings include hyperfluorescence and
hypofluorescence and window defects
corresponding to zones of RPE derangement
• DDx: MEWDS, RP, syphilis, DUSN, CAR
21
Q
Where are Koeppe nodules seen?
A
Koeppe nodules are iris nodules located near the
pupillary margin
22
Q
Where are Busacca nodules seen?
A
Busacca nodules are iris nodules seen in the iris stroma
23
Q
Where are Berlin nodules seen?
A
Berlin nodules are iris nodules seen in the iridocorneal angle
24
Q
What are the risk factors for CMV retinitis and how does the virus infect the retina?
A
• Main risk factors: CD4 count < 50 cells/mm3, severe
systemic immunosuppression
• CMV remains latent in the host and may reactivate
if the host immunity is compromised. It can reach
the retina hematogenously and infect the vascular
endothelium which then spreads to retinal cells
25
What are the risk factors for developing chronic uveitis in juvenile idiopathic arthritis (JIA)?
• Female sex
• Age at onset less than 6 years old
• ANA positivity
• Pauciarticular involvement: less than or equal to 4
joints involved during the first 6 months of disease
26
How does Diffuse Unilateral Subacute Neuroretinitis (DUSN) differ from Ocular Cysticercosis in relation to treatment response and lab work up?
27
What were the main findings and conclusion of the Endophthalmitis Vitrectomy Study (EVS)?
• The EVS studied vitreous tap and inject versus pars
plana vitrectomy and inject in post-phaco eyes
with endophthalmitis
• The results showed that eyes that presented with
hand motion (HM) vision or better vision had
equivalent outcomes with tap and inject or PPV
and inject, but eyes with LP vision or worse had a
better chance of visual recovery after PPV with
injection
• Therefore, PPV with injection is recommended for
eyes with LP or worse
28
What is the pathogen, titer pattern, and ocular findings in infectious mononucleosis?
• Infectious mononucleosis is caused by the Epstein
Barr virus
Titer Pattern:
• Viral Capsid Antigen IgM titers usually rise 4 weeks
post-exposure.
• Early antigen titers rise 6-10 weeks post exposure
and reach their max during the acute phase. They
disappear 6-12 months post infection
• Viral Capsid Antigen IgG may persist for life
Ocular findings:
• Mild follicular conjunctivitis (most common),
dacryoadenitis, keratitis, episcleritis, iridocyclitis,
pars planitis, optic neuritis, macular edema,
macular edema, choroiditis
29
What is the prophylactic treatment for pneumocystis jirovecii?
• Trimethoprim/sulfamethoxazole
30
What pathogen is Fuchs heterochromic iridocyclitis associated with and what are the typical examination findings?
● Associated with rubella virus; other studies have
found association with CMV, toxocara caniis,
toxoplasmosis, HSV
● Typical exam findings include:
○ Heterochromia
○ Iris stromal atrophy
○ Unilateral uveitis
○ Small diffuse KP’s
○ Lack of posterior synechiae
○ Unilateral PSC
31
What is the rate of retinal reattachment after one surgery for RRD associated with uveitis and what causes the increased failure?
• Rate of reattachment in eyes with uveitis is 60%
compared to 90% in eyes without uveitis
• Increased failure is likely due to proliferative
vitreoretinopathy, vitreous disorganization, and
poor visualization during surgery
32
What is the incidence of rhegmatogenous retinal detachment in uveitis patients?
• Incidence of RRD in uveitis is 3%, which is greater
than the general population
• It is especially common in panuveitis and infectious
uveitis (ARN)
33
Which topical steroids produce less rise in IOP?
• Rimexolone
• Loteprednol
• Fluorometholone
34
What medication for intermediate uveitis is contraindicated in patients with multiple sclerosis (MS)?
• TNF inhibitors like infliximab are contraindicated in
MS as they can induce demyelinating disease
35
What is the pathogenesis and prognosis of Subacute Sclerosing Panencephalitis (SSPE)?
• SSPE is the rare late-onset sequelae of primary
measles infection with uveitis without vitritis
• The typical course is primary measles infection
before age 2, then 5-15 years without evidence
of active infection. This is followed by
reactivation of the virus with progressive
neurological decline
• Usually fatal unless caught early and treated
with antiviral agents
36
What is the pathogen that causes cat scratch fever and what is the classic triad of presenting findings?
Pathogen
• Bartonella henselae → a gram negative bacteria
transmitted via scratch, lick, or bite of an infected cat
Classic triad:
• Fever
• Lymphadenopathy
• Neuroretinitis
37
What is the treatment for band keratopathy?
Chelation:
• Remove calcium from Bowman layer
• Remove epithelium → soak cornea with
0.5%-1.5% of disodium
ethylenediaminetetraacetic acid (EDTA) in a
corneal trephine → gentle surface agitation
with a cellulose sponge can enhance release of
calcium
38
The Endophthalmitis Vitrectomy Study (EVS) found which organisms to have the best visual prognosis?
• EVS found coagulase negative staph (S.
epidermidis, S saprophyticus) to have the best
visual prognosis
• 84% of cases had visual acuity of 20/100 or better
39
The Endophthalmitis Vitrectomy Study (EVS) found which organisms to have the worst visual prognosis?
• EVS found enterococci to have the worst visual
prognosis
• 14% of cases had visual acuity of 20/100 or better
40
What two main side effects should be monitored for during cyclosporine use?
• Hypertension
• Nephrotoxicity
41
What are the common indications for rifampin and what is the main side effect of rifampin?
• Commonly used for tuberculosis, leprosy, and
legionnaires disease
• Side effect: Orange discoloration of urine, sweat,
saliva, and tears
42
What is the pathophysiology of Vogt-Koyanagi-Harada (VKH) syndrome and what is the classic presentation?
• VKH is a T cell mediated aggression against
melanocytes of all organ systems in genetically
susceptible individuals
• It affects pigmented structures in the eye, inner
ear, meninges, skin, and hair
• It presents as a chronic, bilateral, diffuse,
granulomatous panuveitis
• Most common in Asian, Asian Indian, Hispanic,
Native American, and Middle Eastern ancestry
43
What are the clinical stages of Vogt-Koyanagi-Harada syndrome?
1. Prodromal stage: flu-like/meningitis-like
symptoms, vertigo, tinnitus, dysacusis, fever,
nausea
2. Acute uveitic stage (few days after prodrome):
blurry vision, bilateral granulomatous anterior
uveitis, vitritis, thickening of choroid, optic disc
hyperemia/edema, multiple serous RD’s
3. Convalescent stage (several weeks later):
resolution of serous RD, sunset-glow fundus
(depigmentation of choroid), perilimbal vitiligo
(Sugiura sign), vitiligo, alopecia, poliosis
4. Chronic recurrent stage (if not treated adequately):
recurrent episodes of granulomatous anterior
uveitis, iris depigmentation, posterior synechiae,
cataract, glaucoma, CNV, subretinal fibrosis. Most
vision threatening
44
What HLA types are associated with Vogt-Koyanagi-Harada Syndrome?
• HLA-DRB1
• HLA-DR4: in Japanese patients
45
What is the treatment for Vogt-Koyanagi-Harada Syndrome?
Acute stage
• Respond to early and aggressive treatment with
steroids
• 1-1.5 mg/kg/day of intravenous
methylprednisolone daily for 3 days followed by
high dose oral steroids
• If intolerant to systemic steroids → intravitreal
steroids
• Systemic steroids are tapered very slowly
according to clinical response, over 6-12 month
period, to prevent chronic recurrent stage,
minimize extraocular manifestations and early
recurrence
• May consider early immune modulating therapy
46
What is the approach to sub-Tenon (Nozik technique) triamcinolone?
• 25 G, ⅝-inch needle
• Bevel against the scleral
• Needle is advanced to the hub with a side-to-side
motion to detect any scleral engagement
• Position tip of the needle in between the Tenon
capsule and the scale
• Preferred location is superotemporal quadrant →
think superotemporal (ST) for sub-Tenon (ST)
• Inferotemporal approach can also be performed in
a similar fashion but with short 27 G needle
47
What is the mechanism of action of NSAIDs?
Inhibition of cyclooxygenase 1 and 2
48
What are the common uveitic conditions with HLA type associations?
• Birdshot: HLA-A29
• Reactive Arthritis: HLA-B27
• Behcet Disease: HLA-B51
• VKH and Sympathetic Ophthalmia: HLA-DR4
• Intermediate Uveitis: HLA-B8, B51, DR2, D15
• Multiple Sclerosis: HLA-B27, DR2
• TINU: HLA-DQ in white North Americans, -DR14 in
Spanish
49
“Refractile bodies” in the aqueous is a classic histologic finding in what cell type and seen in which condition?
• Lipid-laden macrophages
• Seen in phacolytic uveitis
50
What is the pathogen, epidemiology, ocular findings, and treatment for onchocerciasis?
• Onchocerciasis or “river blindness” is caused by the worm
onchocerca volvulus which has a microfilariae form and an
adult worm form
• It is a leading cause of blindness in the world, particularly
sub-Saharan Africa
• Ocular findings:
• Free swimming microfilariae in anterior chamber
• Uveitis
• Secondary glaucoma
• Secondary cataract
• Corneal stromal opacity
• Chorioretinal atrophy
• Optic Atrophy
• Treatment: Ivermectin + Doxycycline
• Ivermectin prevents release of the microfilariae from
pregnant female worms
• Doxycycline induces sterility of the adult worm
51
Which features of herpetic uveitis are helpful diagnostic hallmarks?
● Ocular hypertension
○ Due to trabeculitis
○ Due to inflammatory cells obstructing and
congesting the trabecular meshwork
○ As high as 50-60 mm Hg
● Iris atrophy (patchy or sectoral) seen with
retroillumination
52
What is the differential for hypopyon in non-endophthalmitis uveitis?
• HLA-B27 uveitis
• Behcet disease
• Rifabutin (medication induced, sterile hypopyon)
53
What is the mechanism of action and indication for tocilizumab?
Mechanism of action:
• Monoclonal antibody that acts as an antagonist to
IL-6 receptor
• IL-6 is a proinflammatory cytokine
Indications:
• Juvenile idiopathic arthritis-associated uveitis,
rheumatoid arthritis and other types of uveitis
refractory to other treatments
• FDA-approved for GCA
• Also treats macular edema
54
What is the mechanism of action of Anakinra?
• Anakinra is a recombinant IL-1 receptor antagonist
55
What is the mechanism of action of Adalimumab?
• Adalimumab is a fully human monoclonal IgG1
antibody directed against TNF-alpha
56
What is the mechanism of action of etanercept?
• Etanercept is a TNF receptor blocker
57
What is the ideal dosing for hydroxychloroquine, what is the risk of toxicity over time, and what are important risk factors for retinal toxicity?
● Ideal dosing: 5 mg/kg of real body weight or less
● If used at this dose…
○ < 1% risk of toxicity during first 5 years
○ < 2% risk of toxicity up to 10 years
○ ~20% risk of toxicity after 20 years
● Important risk factors for retinal toxicity:
○ Daily dose > 5 mg/kg of real body weight
○ Duration of use > 5 years
○ Renal disease (cleared by kidney)
○ Tamoxifen use (risk of toxicity increased ~5x;
mechanism unknown)
○ Macular disease (interferes with screening
tests)
58
List the following steroids in order of strongest to weakest potency: dexamethasone 0.1%, prednisolone acetate 1%, difluprednate 0.05%
Difluprednate 0.05% (Durezol)
↓
Dexamethasone 0.1% (Maxidex)
↓
Prednisolone acetate 1% (Pred Forte)
59
What is the mechanism of steroid-induced ocular hypertension, how long does it take to develop, and how long does it take to resolve?
• Steroid causes outflow reduction
• 1/3 population may be steroid responder
• Usually takes 3-6 weeks for response
• Return to pretreatment IOP levels within 10 days
to 3 weeks after discontinuation of steroid
60
Which antibiotics were used in the Endophthalmitis Vitrectomy Study and what was their method of delivery?
• Vancomycin-amikacin given intravitreally
• Vancomycin-ceftazidime given subconjunctivally
• Vancomycin-amikacin given topically
61
What are the contraindications to sub-Tenon steroid injection?
• Infectious uveitis
• Necrotizing scleritis
62
What is the mechanism of action of tacrolimus and cyclosporine and how do they differ?
• Both are potent calcineurin inhibitors that block
T-cell signaling
• Cyclosporine is a product of fungus Beauveria nivea
• Tacrolimus is a production of Streptomyces
tsukubaensis
• Tacrolimus has equal efficacy for chronic uveitis,
but less risk for systemic hypertension
63
What medications are associated with drug-induced uveitis and how is it treated?
• Rifabutin (used for mycobacterium avium)
• Systemic fluoroquinolones, especially moxifloxacin → induce
iris depigmentation and uveitis
• Bisphosphonates
• Sulfonamides
• Diethylcarbamazine (DEC) (used for onchocerciasis)
• Oral contraceptives
• Etanercept (anti-TNF) → new-onset uveitis, systemic
sarcoid-like syndrome
• BCG vaccine, flu vaccine, PPD
• Topical anti-glaucoma medications: metipranolol,
anticholinesterase inhibitors, prostaglandin F2alpha
analogues, brimonidine
• Drugs injected directly into the eye: urokinase, cidofovir,
anti-VEGF
Treatment
• Topical steroids and cycloplegics
• If unresponsive, stop or taper offending medication
64
What are the anatomic types of uveitis listed in order from most to least common?
Anterior uveitis
↓
Panuveitis
↓
Posterior uveitis
↓
Intermediate uveitis
• Anterior uveitis is more likely to be idiopathic
• Posterior uveitis is more likely to be infectious
65
What geographic locations are the following types of uveitis associated with: Behçet's disease, birdshot, VKH, TB, leptospirosis and toxoplasmosis?
• Behçet's: Turkey and China
• Birdshot: Western Europe
• VKH: Asia, American Indian, Mediterranean,
Middle East
• TB and leptospirosis: main causes of infectious
uveitis in India
• Toxoplasmosis: Southeastern Brazil
66
What is the pathogenesis, ANCA type, the affected tissues, mortality, and treatment of granulomatosis with polyangiitis?
• Granulomatosis with polyangiitis is an
autoimmune, large vessel vasculitis that mainly
affects the sinus, lungs, kidneys, eyes, joints, skin,
and CNS
• Orbital involvement is a result of contiguous
spread from nasal sinuses
• 90% of all ANCA is c-ANCA, with specificity to PR3
• Untreated patients have a 1 year mortality rate of
80%
• Treatment: systemic steroids and
immunomodulatory therapy, cyclophosphamide
67
In the Endophthalmitis Vitrectomy Study (EVS), which method of collecting intraocular specimens for culture was associated with increased rate of retinal tears?
• In the EVS, both vitreous tap and vitrectomy were
associated with an increased rate of retinal tears
68
When would azathioprine use not be recommended?
• Low or no thiopurine S-methyltransferase (TPMT)
activity
• Patient taking allopurinol → combination of
allopurinol and azathioprine is high risk for bone
marrow suppression
69
What are the traditional DMARDs?
● The traditional DMARDs are:
○ Methotrexate
○ Sulfasalazine
○ Leflunomide
70
What are the risk factors for post-traumatic endophthalmitis after a ruptured globe?
• Delay in closure > 12 hours
• Contaminated wound
• Retained foreign body (higher risk factor)
• Rupture of posterior capsule during surgery
71
What are the typical pathogens associated with post-traumatic endophthalmitis?
• Bacillus
• S. epidermidis
• Streptococci
• Fungi
• S. aureus
72
Which glaucoma procedure should be avoided in patients with uveitic glaucoma?
• Diode cyclophotocoagulation should be avoided in
uveitis glaucoma because it cause tremendous
amount of additional inflammation and may cause
chronic hypotony and phthisis bulbi
73
What is the glaucoma procedure of choice in uveitic glaucoma?
• The procedure of choice in uveitic glaucoma is a
tube shunt (i.e. Ahmed valve)
• Filtering surgery often fails quickly in the presence
of chronic inflammation
74
What are the best and worst IOL types and ideal location for a patient with history of chronic uveitis?
Best type of IOL
• Hydrophobic, acrylic 1-piece
Best location for IOL
• Posterior chamber (in-the-bag)
IOL type not recommended
• Silicone → associated with increased
inflammation; also silicone oil may be needed for
future retinal surgery
75
Why does ocular toxoplasmosis in HIV/AIDS require extended systemic treatment?
• Toxoplasmosis in HIV/AIDS requires extended
systemic treatment due to high risk of cerebral
involvement
• There is a 56% incidence of cerebral involvement in
susceptible patients
76
What is the classic presentation of toxoplasmosis retinochoroiditis? What are the classic exam findings?
● Classic presentation:
○ Unilateral blurry vision/floaters
● Classic exam findings:
○ Mild to moderate potentially granulomatous
anterior uveitis
○ Focal, white retinitis with overlying vitreous
inflammation = “headlight in the fog”
adjacent to a chorioretinal scar
○ Retinal vessels may show perivasculitis with
diffuse venous sheathing and segmental
arterial sheathing
○ Other findings:
■ CME
■ Cataract
■ Serous retinal detachment
■ Choroidal neovascularization
77
What is the first-line immunomodulatory therapy (IMT) for juvenile idiopathic arthritis (JIA)?
• First-line IMT in JIA is methotrexate
• The goal of therapy is to reduce the dose of
systemic steroid use especially in children due to
the risk of growth retardation with premature
closure of epiphyses and multiple other risks with
chronic oral steroids
78
What is the classic presentation and findings in Tubulointerstitial Nephritis and Uveitis Syndrome (TINU)?
• TINU most commonly presents in adolescent girls
and women up to their early 30s
• Systemic symptoms start before ocular
inflammation and include headache, fever,
malaise, fatigue, weight loss, arthralgias, myalgias,
and flank pain
• Ocular findings: bilateral, nongranulomatous
anterior uveitis
• Renal disease is usually self-limited and
spontaneously resolves
79
What are the criteria for the clinical diagnosis of Tubulointerstitial Nephritis and Uveitis Syndrome (TINU)?
Criteria for clinical diagnosis of TINU:
• Abnormal serum creatinine or decreased
creatinine clearance
• Abnormal urinalysis: increased
beta-2-microglobulin, pyuria, hematuria,
eosinophils, proteinuria, white cell casts,
normoglycemic glycosuria
• Associated systemic illness: fever, weight loss,
anorexia, fatigue, arthralgias, myalgias
• Abnormal liver function, elevated ESR, eosinophilia
80
What HLA type is associated with Tubulointerstitial Nephritis and Uveitis Syndrome (TINU)?
HLA-DRB1*0102
81
What is the treatment and prognosis for Tubulointerstitial Nephritis and Uveitis Syndrome (TINU)?
• The treatment for TINU is prednisone 1 mg/kg/day
for 3-6 months followed by a slow taper
• Most patients recover normal renal function and
baseline visual acuity
82
What are the cytokines produced by T helper 1 (Th1) cells?
● Th1 cells produce:
○ IL-2
○ IL-12
○ IFN-gamma
○ TNF-beta
● Th1 cells also help B cells secrete IgG1 and IgG3,
and inhibit Th2 cells
83
What cytokines are produced by Th2 Cells?
• Th2 cells produce:
• IL-4
• IL-5
• IL-10
• Th2 cells also help B cells secrete IgE and IgA and
inhibit Th1 cells
84
What is the etiology and what are the tissues involved, ocular findings, and treatments for relapsing polychondritis?
Etiology
• Autoimmune disease
• Widespread inflammation of cartilage
Most common tissues involved
• Auricular inflammation
• Arthropathy
• Nasal cartilage inflammation
Ocular manifestations (involved 50% of time):
• Scleritis
• Conjunctivitis
• Uveitis
• Retinal vasculitis
Treatment
• Systemic steroids
• Dapsone
• Methotrexate
• Cyclophosphamide
85
What is the difference between Vogt-Koyanagi-Harada (VKH) and sympathetic ophthalmia (SO)?
VKH:
• History of penetrating ocular trauma or other ocular or
systemic diseases are absent
• Race predilection: Asian, Hispanic, Native American,
Middle Eastern
• Systemic manifestations: poliosis, vitiligo, alopecia,
hearing loss, meningeal signs
• Serous retinal detachment is more common
• Choriocapillaris inflammation present
SO:
• Following intraocular surgery (vitrectomy) or
penetrating trauma
• No race or sex predilection
• Systemic symptoms uncommon
• Serous retinal detachment are less common
• Choriocapillaris spared in early stage
86
What are the ocular signs of Alport Syndrome?
● Alport Syndrome, AKA hereditary nephritis, has the
following ocular signs:
○ Anterior lenticonus
○ Posterior Subcapsular Cataract
○ Posterior Polymorphous Membrane
Dystrophy (PPMD)
87
What is the mechanism of mycophenolate mofetil? How should it be taken? What are the side effects?
• Mycophenolate mofetil is a noncompetitive,
reversible inhibitor of inosine monophosphate
dehydrogenase which inhibits de novo purine
synthesis
• It should be taken on an empty stomach (1 hour
prior to eating or 2 hours after a meal) - taking with
food reduces absorption
• < 20% patients have adverse effects such as GI
upset, diarrhea, leukopenia
88
What are the side effects of cyclophosphamide?
● The side effects of cyclophosphamide include:
○ Hemorrhagic cystitis
■ If microscopic hematuria → increase
water intake
■ If gross hematuria → stop medication
○ Bladder Cancer
○ Sterility
○ Reversible alopecia
89
Which uveitis conditions classically benefit from early steroid-sparing immunomodulatory therapy (IMT)?
• Behçet's
• Sympathetic ophthalmia
• Vogt-Koyanagi-Harada Syndrome
• Necrotizing sclerouveitis
90
What is the treatment for congenital toxoplasmosis?
• The treatment regimen of pyrimethamine,
sulfadiazine, and folinic acid for the first year of life
has been shown to decrease the severity of vision
loss in congenital toxoplasmosis
91
What is Toxic Anterior Segment Syndrome (TASS) and what are the potential causes?
• TASS is postoperative anterior segment inflammation caused
by non-infectious substance that enters ant segment → toxic
damage to intraocular tissues
• Potential causes:
• Bacterial endotoxins or particulate contamination of
BSS
• Intraocular irrigating solutions with abnormal pH,
osmolarity, or ionic composition
• Denatured ophthalmic viscosurgical devices (OVD)
• Intraocular medications (antibiotics in irrigation
solutions or intracameral antibiotics)
• Topical ointment
• Inadequate sterilization of surgical instruments and
tubing
• Inadequate flushing of instruments between cases
resulting in build up of OVD
• Preservatives in medications used intraoperatively
• Metallic precipitate
• Starts within 24 hours of cataract surgery (infectious
endophthalmitis 2-7 days after surgery)
92
What is the treatment and prognosis of Toxic Anterior Segment Syndrome (TASS)?
• TASS may result in permanent iris damage →
dilated pupil or irregular pupil that constricts and
dilates poorly; potential trabecular meshwork
damage
• Improves with steroids
93
What considerations should be taken for cataract surgery in a juvenile idiopathic arthritis (JIA) patient?
● For cataract surgery in a patient with JIA:
○ The patient should be without active
inflammation and macular edema for at least
3 months before surgery
○ Anterior capsulorhexis should be between
5-6 mm
○ An acrylic lens should be placed in the bag
○ Primary posterior capsulotomy should be
performed at the time of surgery
94
What patterns of distribution are most typical for CMV retinitis?
• Perivascular distribution is most typical for CMV
since the virus initially infects the endothelium of
blood vessels
• If retinitis is posterior, it typically has a fulminant
or hemorrhagic pattern
• If it is more peripheral it typically has a granular
pattern with less hemorrhage
• Frosted branch retinitis is another classic pattern
95
What is the classic triad of measles? What other ocular problem can measles cause?
• Classic triad, the three C’s: Cough, Coryza (runny
nose), Conjunctivitis
• Systemic symptoms begin a few days before rash
which starts at the head and then spreads down
• Measles retinopathy: significant vision loss 1 week
after rash onset. Associated with retinal edema,
macular star, optic disc swelling, narrowed
arterioles, intraretinal hemorrhages. As the
retinopathy subsides, pigmentary retinopathy
resembling RP (bone spicules) or rubella (salt and
pepper) can be left. It is associated with permanent
VF loss
96
What are the most common bacterial causes of endophthalmitis in each of the following scenarios: acute postoperative, chronic postoperative, cutaneous infection, endocarditis, liver abscess, bleb-associated?
• Acute postoperative: coagulase negative
Staphylococcus, Streptococcus species,
gram-negative organisms
• Chronic postoperative: Propionibacterium acnes,
Staphylococcus epidermidis, Corynebacterium
species
• Cutaneous infection: Staphylococcus aureus
• IVDU: Bacillus species
• Endocarditis: Streptococcus species
• Liver abscess (especially in Asia): Klebsiella
• Bleb-associated: Streptococcus species,
Haemophilus species, gram-positive organisms
97
What characterizes lupus retinopathy?
• Lupus retinopathy is associated with retinal
occlusive disease (i.e. BRVO)
• Retinal occlusive disease in lupus is more common
with CNS involvement and presence of
antiphospholipid antibodies
• CNS lupus: causes seizures and strokes
98
Above what threshold daily dose of prednisone should immunomodulatory therapy (IMT) be used for chronic uveitis?
• IMT should be used if ≥ 10 mg PO prednisone is
required for control of chronic uveitis
99
How frequently should Juvenile Idiopathic Arthritis (JIA) be examined?
• Follow up for JIA is based on risk stratification
• JIA Risk factors: ANA+, 6 years or less at diagnosis
of JIA, duration of JIA 4 years or less in patients
with pauci/oligoarticular JIA
• High risk = 3/3 risk factors
• q3 months
• Moderate risk = 2/3 risks
• q6 months
• low risk = 1/3 risks
• q12 months
100
What is the risk for developing chronic iridocyclitis based on joint involvement in Juvenile Idiopathic Arthritis (JIA)?
• Still disease (minimal joint involvement) → rare
development of chronic iridocyclitis
• Polyarticular → ~10% will develop chronic
iridocyclitis
• Pauciarticular → ~85% will develop chronic
iridocyclitis
101
What are the ocular manifestations and systemic manifestations of reactive arthritis? How frequently are patients HLA-B27 positive?
● "Cant see, can’t pee, cant climb a tree"
● Ocular manifestations:
○ Mucopurulent and papillary conjunctivitis (most
common)
○ Iritis
○ Punctate, subepithelial keratitis, or both
● Systemic signs:
○ Keratoderma blennorrhagicum
(papulosquamous rash)
○ Balanitis
○ Sacroiliitis
○ Plantar fasciitis
○ Achilles tendonitis
● Typically follows bout of dysentery (Shigella,
Salmonella, Yersinia, Chlamydia, Ureaplasma)
● 90% are HLA-B27 positive
102
What are the differences between Progressive Outer Retinal Necrosis (PORN) and Acute Retinal Necrosis (ARN)?
● PORN
○ Immunocompromised (CD4 < 50)
○ Absence of vitritis
○ Retinal vasculature minimally involved
○ Posterior pole involved early
● ARN
○ Immunocompetent
○ Presence of vitritis
○ Vasculitis
○ Posterior pole typically spared initially
103
What is the most common type of primary CNS lymphoma?
• Non-Hodgkin B Cell Lymphoma makes up > 90% of
primary CNS lymphomas
104
What immunomodulatory therapy causes drug-induced lupus? What are the clinical findings and what is the prevention and treatment of drug-induced lupus?
• Infliximab → autoantibodies causing lupus-like
syndrome
• After extended period of time the effects include:
• Vascular thrombosis
• Congestive heart failure
• ANA positive
• Antibodies against infliximab which decrease
its effectiveness
• MTX or azathioprine can be used to prevent
development of autoantibodies and antibodies
against infliximab
• Other causes of anti-TNF-alpha associated drug
induced lupus: etanercept and adalimumab
• Treatment: withdrawal of drug → symptoms
resolve within 3 weeks to 6 months
105
What are the most important characteristics to monitor when assessing the response of CMV retinitis lesions to antiviral therapy?
• Lesion size and degree of activity at the border of
the lesion are most important factors to monitor
for response to therapy
106
What are the presenting symptoms, ocular findings, and etiologic pathogens in Whipple disease? How do you diagnose Whipple disease?
• Whipple disease presents with GI symptoms and
migratory polyarthralgias
• Ocular findings include bilateral panuveitis and
retinal vasculitis
• Associated pathogens are tropheryma whipplei
and actinomycetes bacterium
• Diagnosis by duodenal biopsy which shows PAS
positive organisms in macrophages within the
intestinal villi
107
What is the treatment for ocular syphilis?
• Treat as neurosyphilis:
1. 18-24 million units of intravenous aqueous
crystalline penicillin G daily for 10-14 days
2. If compliant, 2.4 MU/day of intramuscular
procaine penicillin + probenecid 500 mg qid
for 10-14 days
• #1 is preferred option to ensure adequate
intraocular levels
• Most recommend initial treatment with #1
followed by intramuscular benzathine penicillin G
2.4 MU weekly for 3 weeks
108
What is the threshold CD4 count below which HIV patients typically become symptomatic? What is the CD4 count range in the asymptomatic phase of HIV? What is the CD4 count range in a healthy adult?
• CD4 count threshold for symptomatic HIV: < 200
cells/mm³
• CD4 count in asymptomatic phase of HIV: 200 to
750 cells/mm³
• CD4 count in healthy adults: 600 to 1500 cells/mm³
109
What is the mechanism of action of cromolyn sodium?
• Cromolyn sodium inhibits degranulation and
stabilizes mast cells and therefore inhibits the
release of mediators of inflammation
• Mast cell degranulation is triggered when IgE
molecules covering the surface of mast cells are
bridged by divalent antigen
• Full efficacy of cromolyn sodium is reached 5-14
days after therapy → not useful for acute
symptoms
110
How does ocular toxoplasmosis differ in AIDS patients versus immunocompetent patients?
● Ocular toxoplasmosis in AIDS:
○ Lesion arises de novo, without associated
chorioretinal scar (newly acquired or spread
from non-ocular site)
○ Multifocal retinochoroiditis more common
○ Size of lesion larger
○ Less overlying vitreous inflammation
● Ocular toxoplasmosis in immunocompetent
patients:
○ Reactivation at site of old chorioretinal scar
(reactivation of congenital infection)
111
What is the treatment for ocular toxoplasmosis in the setting of sulfa allergy (SJS reaction to sulfa drugs)?
● Treatment options for ocular toxoplasmosis in the
setting of a sulfa allergy:
○ Oral clindamycin alone
○ IVI clindamycin plus dexamethasone
○ Azithromycin alone or in combination with
pyrimethamine
112
What are the treatment options for ocular toxoplasmosis in patients without sulfa allergies?
• Triple therapy: pyrimethamine, sulfadiazine and
folinic acid with prednisone added 48 hours after
antibiotics coverage
• Triple sulfa: sulfadiazine, sulfamerazine,
sulfamethazine
• Bactrim DS (trimethoprim-sulfamethoxazole)
• Oral clindamycin (sole antibiotic)
• IVI clindamycin + dexamethasone
• Azithromycin alone or combined with
pyrimethamine
• Doxycycline
• Minocycline
• Atovaquone
113
What is the cause of Progressive Outer Retinal Necrosis (PORN)? What are the exam findings? What is the treatment?
• PORN is a variant of necrotizing herpetic retinitis
caused by VZV (most common) or HSV (rare) in
immunocompromised patients
• Exam shows retinal necrosis with lack of vitritis and
minimal retinal hemorrhages. It involves the
macula early on and lacks vasculitis. It also lacks
the granular border of CMV lesions
• Treatment: intravitreal and systemic ganciclovir or
foscarnet
114
What are the screening and confirmatory tests for HIV?
• HIV screening test: Antibody detection by ELISA
• HIV confirmatory test: Western blot
115
What examination techniques does the Standardization of Uveitis Nomenclature (SUN) recommend in order to accurately grade anterior chamber inflammation?
• To grade anterior chamber reaction you count the
number of cells seen in a 1 mm x 1 mm high
powered light beam at full intensity at 45-60
degree angle in a dark room
116
What is the Standardization of Uveitis Nomenclature (SUN) scale for grading cell?
• < 1 cell = 0 grade
• 1-5 cells = 0.5+ grade
• 6-15 cells = 1+ grade
• 16-25 cells = 2+ grade
• 26-50 cells = 3+ grade
• > 50 cells = 4+ grade
117
What is the most common ocular manifestation of congenital rubella syndrome? What are the other associated ocular findings? What is the cause of poor vision in these patients?
• The most common ocular manifestation of
congenital rubella syndrome is pigmentary
retinopathy
• Other ocular findings:
• Cataract
• Microphthalmos
• Glaucoma
• Most common cause of poor vision is cataract and
microphthalmos. Retinopathy usually doesn’t
affect the vision
• Systemic manifestations of congenital rubella
syndrome:
• Deafness (most common)
• Cardiac malformations (patent ductus
arteriosus)
• Ocular findings
118
What cytokine is elevated in the vitreous in intraocular lymphoma?
• IL-10 is elevated in intraocular lymphoma
• This is due to malignant B lymphocytes that
produce this cytokine in greater proportions
119
What cytokine is elevated in the vitreous in inflammatory uveitides?
• IL-6 is elevated in inflammatory uveitides
120
Among which racial groups is VKH least common?
• VKH is least common among Caucasians and Africans
121
What is the rate of endophthalmitis after PPV?
• Rate of endophthalmitis after PPV is 1 in 2000
• Rates are higher in diabetics and in retained
intraocular foreign bodies
122
What is the rate of bleb-related endophthalmitis after filtering surgery?
• The rate of bleb-related endophthalmitis after
filtering surgery ranges from 1.3% per patient year
for superior blebs to 7.8% per patient-year for
inferior blebs
123
What was the purpose of the ESCRS endophthalmitis study? What were the findings?
• The ESCRS endophthalmitis study examined the
potential benefit of intracameral antibiotics
(cefuroxime) instilled at the end of cataract surgery
in preventing post-op endophthalmitis
• It found that intracameral antibiotics reduced the
risk of endophthalmitis 5-fold
• The incidence of post-op endophthalmitis in eyes
receiving intracameral antibiotics was 0.07%
124
What type of Behçet's has the highest mortality?
• Neuro-Behçet's has the highest mortality with a
mortality rate up to 10%
• Neuro-Behçet's presents with headaches, strokes,
CN palsies, and dementia
125
What is the presentation and histology of Juvenile xanthogranuloma?
• Juvenile xanthogranuloma presents before age 1
with benign iris lesions that can cause spontaneous
hyphemas and reddish-yellow skin lesions
• The pathology is non-Langerhans cell histiocytosis
with Touton giant cells with large, foamy
histiocytes
Intraocular
126
Large eosinophilic intranuclear or intracytoplasmic inclusion bodies are highly specific to what type of infection?
• Large eosinophilic intranuclear or intracytoplasmic
inclusion bodies (Owl’s Eyes) are highly specific of
CMV infection
127
How do you describe Langhans giant cells and in what type of disease process are they usually found?
• Langhans giant cells are formed by a fusion of
epithelioid cells with their nuclei arranged in a
horseshoe shape
• They occur in many granulomatous conditions,
most notably in TB
128
What ocular problems can be associated with pars planitis?
● Pars planitis is typically bilateral (80% of the time),
but may be asymmetric
● Other ocular problems that can be associated with
pars planitis include:
○ CME (most common cause of decreased
vision)
○ Spontaneous vitreous hemorrhage
○ Tractional and rhegmatogenous retinal
detachments
129
How long does Retisert (fluocinolone acetonide) intravitreal implant work, what is it used for, and what are the associated side effects?
● Retisert releases steroid for a median period of 30
months
● It is indicated in chronic non-infectious posterior
uveitis
● Side effects include:
○ All phakic patients will develop a cataract
○ 30% will require glaucoma filter after 2 years
of implantation
130
What is the pathophysiology of a Type V hypersensitivity reaction and what are some examples?
• Type V hypersensitivity reactions are caused by
stimulatory hypersensitivity antibodies that react
with specific cell surface receptors and depress or
stimulate cell function
• Examples include Graves disease and myasthenia
gravis
131
What is the pathophysiology of a Type I hypersensitivity reaction and what are some examples?
• Type I hypersensitivity reactions are caused by
pre-formed IgE antibodies bound on mast cell
receptors that react to the offending agent
• Binding causes degranulation of the mast cell and
histamine release
• Examples include anaphylactic, allergic, or atopic
reactions
132
What is the pathophysiology of a Type II hypersensitivity reaction and what are some examples?
• Type II hypersensitivity reactions are caused by
antibodies that bind to the host antigens (cell
surface components) and activate complement
• Example is ocular cicatricial pemphigoid
133
What is the pathophysiology of a Type III hypersensitivity reaction and what are some examples?
• Type III hypersensitivity reactions are caused by
immune-complex deposition in tissues which
activate complement and other effector systems
• Examples include serum sickness, SLE, and RA
134
What is the pathophysiology of a Type IV hypersensitivity reaction and what are some examples?
• Type IV hypersensitivity reactions are delayed-type
hypersensitivity reactions due to the recruitment
of pre-sensitized immune cells. These reactions are
not antibody mediated.
• Examples include Stevens-Johnson Syndrome,
contact dermatitis, and positive PPD testing
135
What medication is used to reverse methotrexate toxicity?
• Leucovorin calcium, folinic acid is a reduced form
of folic acid that is used to rescue individuals from
methotrexate toxicity
136
Which type of endophthalmitis is associated with the worst visual prognosis?
• Post-traumatic endophthalmitis is associated with
the worst visual prognosis
• Approximately 10% of patients with post-traumatic
bacterial endophthalmitis will have final VA 20/400
or better
• Approximately 25% of cases of post-traumatic
endophthalmitis are caused by bacillus cereus and
has a fulminant course that often leads to loss of
the eye
• Endophthalmitis occurs in approximately 5% of
penetrating injuries and has a higher incidence if
intraocular foreign body is involved or if it occurs in
a rural setting
137
What is the prognosis of punctate inner choroidopathy (PIC)?
• PIC is usually self-limited, but recurrences are common
• Visual prognosis is good in absence of CNV
138
Are patients with inflammatory bowel disease and sclerouveitis typically HLA-B27 positive or negative?
• Patients with inflammatory bowel disease and
sclerouveitis are typically HLA-B27 negative
• They can also have joint pain that resembles RA
and do not develop sacroiliitis, unlike IBD with
acute iritis
139
Are patients with inflammatory bowel disease and acute iritis typically HLA-B27 positive or negative?
• Patients with inflammatory bowel disease and
acute iritis are typically HLA-B27 positive and
develop sacroiliitis
140
What is the first-line treatment of cystoid macular edema (CME) in pars planitis?
• Sub-Tenon steroid injection is first-line for CME in
pars planitis
141
What are the seronegative spondyloarthropathies?
• Ankylosing spondylitis
• Reactive arthritis
• Inflammatory bowel disease
• Psoriatic arthritis
• These are all strongly associated with HLA-B27 and
may feature spondylitis and sacroiliitis
142
What are the typical classes of medication used to treat HIV?
• Nucleoside reverse transcriptase inhibitors (NRTI)
• Non-nucleoside reverse transcriptase inhibitors
(NNRTI)
• Protease inhibitors (PI)
• Fusion inhibitors
• Entry inhibitors
• Integrase strand transfer inhibitors
143
What are the presenting symptoms, lab results, imaging findings in birdshot retinochoroidopathy?
• Birdshot retinochoroidopathy, AKA vitiliginous
chorioretinitis, is an inflammatory condition that
presents most commonly in caucasian women past the
4th decade of life with decreased vision, floaters,
nyctalopia, dyschromatopsia, glare, photopsias,
bilateral vitritis, cream colored depigmented lesions ¼
to ½ DD in size, most numerous in posterior nasal
fundus and around the optic nerve radiating towards
the periphery
• ICG shows multiple hypofluorescent spots more
numerous than those apparent on slit lamp
examination or IVFA
• ERF shows prolonged 30 Hz flicker implicit times,
diminished B wave (abnormal Muller and bipolar cells)
compared to A wave amplitude
• It is incompletely responsive to steroids alone and
requires early IMT
144
What is the cause of delayed-onset (chronic) endophthalmitis and what are the signs and work up?
● Delayed-onset (chronic) endophthalmitis is
typically caused by propionibacterium acnes
● Ocular signs include
○ Granulomatous KPs
○ Small hypopyon
○ Mild vitritis
○ Appearance of posterior capsule opacity
● Work up:
○ Cultures, IVI antibiotics
○ If not responsive to IVI antibiotics, vitrectomy
with partial or entire capsular bag removal
and IOL removal are usually the next step
145
What are the predominant symptoms of intermediate uveitis?
• Floaters and blurred vision are the predominant
symptoms of intermediate uveitis
146
What are the three classic presentations of ocular toxocariasis? How is toxocariasis diagnosed?
● Ocular toxocariasis presents in 1 of 3 ways:
○ Peripheral granuloma (50% of cases)
○ Localized macular granuloma (25% of cases)
○ Leukocoria (25% of cases)
● Diagnosis is by clinical features and supported by
anti-toxocara antibiotics (serum and ocular fluid),
peripheral eosinophilia)
147
What is immune recovery uveitis (IRU) and how does it present?
• IRU occurs in patients with CMV retinitis who
recover their immune status through use of HAART
therapy
• It presents with anterior or intermediate uveitis
and CME
• CME can be resistant to treatment
• Cidofovir causes > 10-fold risk of developing IRU
148
What are the types of pauciarticular Juvenile Idiopathic Arthritis (JIA)?
● Type 1 Pauciarticular JIA:
○ Girls < 5 years old, ANA positive, chronic
uveitis
● Type 2 Pauciarticular JIA:
○ Older boys that end up developing HLA-B27
related uveitis, acute and recurrent uveitis
149
How do you diagnose primary intraocular lymphoma (PIOL)?
● Gold standard for diagnosis of PIOL:
○ Cytological identification of lymphoma cells
within the eye via vitreous, retinal, or
subretinal biopsy
○ Biopsy shows large, pleomorphic, scant
basophilic cytoplasm and large nuclei
○ It has a high false negative rate
● Cytokine analysis will show high levels of IL-10
○ IL-10:IL-6 ratio > 1 is suggestive of PIOL
● Molecular analysis (PCR)
● Immunophenotyping: abnormal immunoglobulin
kappa or lambda light-chain predominance
150
What are the risk factors, symptoms and treatment of leptospirosis?
● Risk factors for leptospirosis:
○ Exposure to natural reservoir for leptospira:
livestock, horses, etc
○ Being in tropical environment
○ More common in Hawaii, accounts for > 50%
USA cases
● Initial disease:
○ 1 month after incubation phase
○ Patient develops acute fever, chills, vomiting,
diarrhea, muscle aches
● Severe septicemic leptospirosis:
○ Severe liver and renal dysfunction
● Caused by a spirochete so false positive RPR or
FTA-antibodies are possible
● Treatment: intravenous penicillin G for 1 week
151
What is the definition of iridocylitis?
• Iridocyclitis is defined as anterior chamber
inflammation which spills over into the retrolental
space
152
What are the contraindications to infliximab?
● Contraindications to infliximab include:
○ SLE due to the risk of lupus-like syndrome (as
with all TNF-alpha inhibitors)
○ Clinically significant active infection
○ History of drug hypersensitivity
○ Heart failure
○ If occult TB, infliximab use is possible with
concurrent TB therapy
153
What is the pathophysiology of posterior synechiae?
• In posterior synechiae, the pigmented epithelium
of the iris is stimulated to migrate over the surface
of the lens capsule, and subsequent adhesion
between the epithelial cells occurs
154
What are the respective vectors and diseases pertaining to the following pathogens: onchocerca volvulus, leishmania, leptospira interrogans?
• Onchocerca volvulus: female black fly, endemic in
sub-Saharan Africa; onchocerciasis/river blindness
• Leishmania: sandflies, leishmaniasis
• Leptospira interrogans: infected when in contact
with animal urine, high risk for sewer workers,
veterinarians, farmers
155
How do you interpret a PPD?
● Positive test is based on exposure:
○ > 5 mm is positive if:
■ Exposed to active TB, HIV infection,
radiographic evidence of healed TB
lesions
○ > 10 mm is positive if:
■ Diabetes, renal failure, systemic
immunomodulatory therapy,
immigrants from TB-endemic areas,
healthcare workers
○ > 15 mm is positive for everyone
156
What are the respective targets of infliximab, adalimumab, and cyclosporine/tacrolimus?
• Infliximab: chimeric mouse and human monoclonal
antibodies that bind TNF-alpha; the murine portion
of the molecule causes infusion reactions
• Adalmumab: humanized TNF-alpha inhibitor
• Cyclosporine/tacrolimus: calcineurin inhibitor;
inhibits IL-2 which regulates T-cell activation
157
Who is at highest risk for osteoporosis?
• Postmenopausal women and men older than 50
years taking 7.5 mg/day or more of steroids for
longer than 3 months
158
What is the differential for pars planitis/intermediate uveitis?
• Sarcoidosis
• Syphilis
• Toxocariasis
• Lyme Disease
• Multiple Sclerosis
159
What are the treatment options for Lyme disease?
• Oral Amoxicillin 500 mg TID
• Oral Doxycycline 100 mg BID
• Oral Cefuroxime 50 mg BID
• Selected macrolides
• IV Ceftriaxone 2 g IV daily
• IV Penicillin G 18-24 million U per day IV divided q4
hours
160
What are the stages of Lyme Disease and their associated ocular involvement, respectively? What testing should be performed if there is severe intraocular inflammation?
• Stage 1: local disease, follicular conjunctivitis
• Stage 2: disseminated disease, uveitis, anterior,
intermediate (most common), posterior, panuveitis
associated with granulomatous anterior, papillitis,
choroiditis, vasculitis, exudative RD
• Stage 3: persistent disease, keratitis, uveitis less
commonly
• Severe intraocular inflammation should be
considered a sign of CNS involvement, thus lumbar
puncture should be obtained
• Treatment: oral amoxicillin or doxycycline; IV
ceftriaxone (for CNS involvement)
161
What are the stages of syphilis and their associated ocular involvement?
• Primary: 3-4 weeks after exposure, painless
chancre at inoculation site
• Secondary: 4-8 weeks after primary chancre, rash,
condyloma late, systemic symptoms
• Latent: early is < 1 year after infection, late is > 1
year after infection
• Tertiary: 1-10 years after infection, ocular syphilis,
neurosyphilis, cardiovascular problems, gumma
(granulomas, rare)
162
What are the ocular complications of HIV?
• HIV retinopathy (most common, 70% of AIDS)
• Opportunistic viral/bacterial/fungal infections
• Kaposi sarcoma
• Lymphomas of retina/adnexa/orbit
• Squamous cell carcinoma of the conjunctiva
163
What are the classic findings of Sjogren Syndrome?
• Keratoconjunctivitis sicca
• Xerostomia (decreased parotid flow rate)
• Lymphocytic infiltration in salivary glands on biopsy
• Labs: positive ANA, RF, SS-A, SS-B
164
What is the virology of the Chikungunya virus and what ocular problems does it cause?
• The Chikungunya virus is a single-stranded RNA
virus from the genus Alphavirus, family Togaviridae
• Spread to humans by infected mosquito
• Causes anterior uveitis and retinitis
(focal/multifocal/confluent retinitis)
165
What are the causes of blind spot enlargement?
• Papilledema
• Chorioretinal inflammatory conditions (i.e.
MEWDS, MFC)
• Acute idiopathic blind spot enlargement
166
What are the causes of cecocentral scotomas (a blind spot between the physiologic blind spot and the point of fixation)?
• Toxic optic neuropathy
• Nutritional optic neuropathy
• Leber hereditary optic neuropathy
167
What is sympathetic ophthalmia, its presentation, and the most common cause?
• Thought to be caused by penetrating injuries or
intraocular surgery
• Granulomatous panuveitis that is bilateral and
asymmetric (more severe in injured eye)
• Presentation: Mutton fat KP’s, difficulty with
accommodation, photophobia, iris thickening,
posterior synechiae, elevated IOP, mid-equatorial
yellow white lesions (Dalen-Fuchs nodules), exudative
retinal detachments, and retinal perivasculitis
• It spares the choriocapillaris
• Earliest stages the choroid has accumulation of
eosinophils
• Most common cause is PPV (0.12% of PPV’s)
• 90% of cases occur within 1 year of injury, but range is
2 weeks to 50 years
168
What is the pathology in the Autoimmune Regulator (AIRE) deficient mice model of uveitis?
• In AIRE Deficient Mice self-tolerance does not
occur in the thymus because they lack the
necessary transcription factor
• Therefore autoreactive T cells are not deleted and
the mice develop autoimmunity that causes a
posterior uveitis
169
What are the respective fundus autofluorescence patterns associated with multifocal choroiditis, Acute Zonal Occult Outer Retinopathy (AZOOR), and Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)?
• Multifocal choroiditis: multiple
hypoautofluorescent spots that correspond with
the chorioretinal atrophy
• AZOOR: central hypoautofluorescence with
peripheral hyperautofluorescence
• APMPPE: Hyperautofluorescent areas correspond
with blockage on the IVFA
170
What is the epidemiology, presenting symptoms, exam findings, IVFA findings, and prognosis for Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)?
• APMPPE prevalence is equal among men and women
• Patients present with loss of vision, central or
paracentral scotomas, and photopsias, usually
preceded by a viral prodrome
• Exam findings include large 1-2 DD yellow-white
placoid lesions in the posterior pole
• IVFA shows early hypofluorescence of lesions, late
staining lesions involute over several weeks and leave
behind significant RPE alterations
• Associated with HLA-B7, DR2
• Associated with immune-driven vasculitis → cerebral
vasculitis
• Prognosis is good with most patients recovering 20/40
or better vision with residual defects
• Of note the fundus appearance is indistinguishable
from syphilis
171
What are the side effects of cyclosporine?
• Hypertension
• Nephrotoxicity
• Gum hyperplasia
• Hirsutism
172
What are two syndromes seen in sarcoidosis and what are they characterized by?
● Lofgren Syndrome:
○ Erythema nodosum, febrile arthropathy,
bilateral hilar adenopathy, acute iritis
○ Responsive to systemic steroids
○ Good prognosis
● Heerfordt syndrome
○ AKA uveoparotid fever
○ Uveitis, parotitis, fever, facial nerve palsy
173
What are the two antineutrophil cytoplasmic antibodies and what disease is each associated with?
● c-ANCA/proteinase3 ANCA
○ Associated with granulomatosis with
polyangiitis
● p-ANCA/myeloperoxidase ANCA
○ Associated with microscopic polyangiitis or
Churg-Strauss
174
What is the biochemical description of antibodies and where/how are they produced?
• Antibodies are glycoproteins that are produced in
lymph nodes and areas of inflammations
• They are produced by B cells that are stimulated to
mature into plasma cells after exposure to an
antigen
• The specificity is determined by the gene
rearrangement of B cells
175
What is the classic triad of ocular histoplasmosis?
• Peripapillary atrophy
• Multiple atrophic punched out chorioretinal scars
• CNVM
176
What is varicella zoster sine herpete?
• Varicella zoster sine herpete is anterior uveitis secondary to VZV without skin lesions
177
What is the pathology of episcleritis?
• Pathology of episcleritis shows palisading
arrangement of epithelioid histiocytes around a
central core of necrotic collagen
178
What is the pathology of scleral nodular fasciitis?
• The pathology of scleral nodular fasciitis shows
activated spindled fibroblasts loosely arranged in
short fascicles with prominent capillary network
179
What are the risk factors for ocular involvement in Candidemia?
● Risk factors for ocular involvement in Candidemia:
○ Hospitalization
○ Recent GI surgery
○ Bacterial sepsis
○ Systemic antibiotics
○ Indwelling catheter
○ Hyperalimentation
○ Debilitation disease
○ Immunomodulatory therapy
○ Prolonged neutropenia
○ Organ transplantation
180
What is the most common causes of bleb-associated endophthalmitis?
• Streptococcus or Haemophilus species
181
What are the geographic associations with histoplasma and coccidioides?
• Histoplasma: Ohio and Mississippi River Valleys
• Coccidioidomycosis: San Joaquin Valley, Southwest
states, Mexico, Central & South America
182
What are the microscopic appearances of aspergillus, cryptococcus, candida albicans, and mucormycosis?
• Aspergillus: septate, dichotomously branching
hyphae
• Cryptococcus: round capsules with halo on India
Ink
• Candida albicans: budding yeast, pseudohyphae
• Mucormycosis: nonseptate
183
What is Diffuse Unilateral Subacute Neuroretinitis (DUSN) and the most common causative organisms?
• DUSN is a multifocal chorioretinitis caused by a
nematode
• Exam shows vitritis, optic nerve swelling, multifocal
inflammation of retina and RPE
• Inflammatory lesions are multifocal, gray-white
color, around 1 DD in size; sometimes s-shaped
nematode is visible in subretinal space
• Definitive treatment: direct photocoagulation of
organism
• Common organisms: ancylostoma canine (dog
hookworm), baylisascaris procyonis (raccoon
roundworm, T canis)
184
How much of blindness in the US is due to inadequately treated uveitis?
• 10% of blindness in the US is due to inadequately treated uveitis
185
What factors actively contribute to immunologic privilege of the transplanted corneal tissue?
• Absence of blood or lymphatic channels in the
graft and graft bed
• Absence of MHC class II antigen-presenting cells
(APCs) in graft
• Reduced expression of MHC-encoded alloantigens
on graft cells
• Increased expression of Fas ligand, which
stimulates apoptosis of killer T cells
• Immunosuppressive cytokines in aqueous humor,
including TGF-B2, vasoactive intestinal peptide,
and a-melanocyte stimulating hormone
186
Which serotypes of C. trachomatis are associated with trachoma, adult and neonatal inclusion conjunctivitis, and lymphogranuloma venereum, respectively?
• Trachoma: serotypes A-C
• Adult and neonatal inclusion conjunctivitis:
serotypes D-K
• Lymphogranuloma venereum: serotypes L1, L2,
and L3
187
What are the treatment options for chlamydial conjunctivitis?
• Azithromycin 1 g PO single dose
• Doxycycline 100 mg PO BID x 7 days
• Tetracycline 250 mg PO QID x 7 days
• Erythromycin 500 mg PO QID x 7 days
188
What is the differential diagnosis for cicatrization?
• Autoimmune: mucous membrane pemphigoid
(MMP, AKA Ocular Cicatricial Pemphigoid), lichen
planus
• Inflammatory: Stevens-Johnson Syndrome
• Infectious: trachoma, herpes zoster
• Surgical: enucleation, posterior approach ptosis
repair, transconjunctival surgery
• Traumatic: chemical/therapy injury
• Medications: miotics
189
What is the classification of vasculitides based on vessel size?
• Large Vessel: Giant Cell Arteritis, Takayasu arteritis
• Medium Vessel: Polyarteritis nodosa, Kawasaki
disease
• Small Vessel: Granulomatosis with polyangiitis
• All sizes: Behçet's disease
190
What is the classic triad of Behcet disease?
• Recurrent aphthous oral ulcers (most common)
• Genital ulcers
• Recurrent uveitis
191
What testing is necessary before starting azathioprine?
• Before starting azathioprine, patients need to be
tested for thiopurine methyltransferase (TPMT)
activity
• TPMT is an important enzyme in the metabolism of
azathioprine
• Patients with deficient TPMT have a high risk of
severe myelosuppression
192
When is treatment of toxoplasmosis relatively indicated?
• Lesions threatening optic nerve or fovea
• Decreased visual acuity
• Lesions associated with moderate to severe
vitreous inflammation
• Lesions greater than 1 disc diameter in size
• Persistence of disease for more than 1 month
• Frequent recurrences
• Presence of multiple active lesions''
193
What is the differential diagnosis for elevated IOP and uveitis?
• Herpetic
• Toxoplasmosis
• Syphilis
• Sarcoidosis
