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Question 1 of 130
A 27-year-old man presents with a history of floaters and blurred vision OD. The patient immigrated to the United States
from India within the last year, is currently in good health, and denies any history of drug use. Two years ago, while in India,
he had a positive tuberculin skin test, and received 9 months of prophylactic isoniazid, rifampin, and ethambutal therapy.
Anterior segment examination is normal. The right fundus is shown in the figure. The left fundus showed findings similar to
those of the right. Vitreous hemorrhage is noted in the inferior vitreous OD. No pars plana snowbanks are noted in either
eye. Which of the following would be least helpful in the workup of this patient?
Serum glucose
Hemoglobin electrophoresis
HLA-A29
Please select an answer
Feedback: This patient has bilateral retinal neovascularization, which may arise from a number of conditions, including
diabetes mellitus, branch retinal vein occlusion (BRVO), sickle-cell retinopathy, Eales' disease, or from inflammatory
conditions such as sarcoidosis, tuberculosis (TB), Behcet's disease, peripheral uveitis, or systemic lupus erythematosus
(SLE). Workup should be directed toward these diagnoses and should include a serum glucose and/or glucose tolerance
test, fluorescein angiography (for BRVO), hemoglobin electrophoresis (for sickle-cell retinopathy), chest xray (for TB,
sarcoid), ACE titer (for sarcoid), ANA titer (for SLE), HLA-B5 (for Behcet's disease), and a tuberculin skin test with anergy
panel (for TB and sarcoid). HLA-A29 would not need to be tested in this patient, because it is associated with birdshot
retinochoroidopathy, which produces a retinochoroiditis, but not retinal neovascularization.
Question 2 of 130
A patient presents with meningismus, vitiligo, and bilateral exudative retinal detachments. What diagnosis is most consistent
with these findings?
Vogt-Koyanagi-Harada syndrome
The patient's laboratory and retinal findings support the diagnosis of Eales' disease.
Unlike with proliferative diabetic retinopathy, in which neovascularization of the iris may develop, this patient has little
chance of ever developing iris neovascularization.
The retinal neovascularization in this patient is likely to remain stable; therefore, treatment is not indicated.
Please select an answer
Feedback: Eales' disease produces an obliterative perivasculitis that leads to retinal ischemia, retinal neovascularization,
vitreous hemorrhage, and, later, tractional retinal detachment and iris neovascularization. Retinal nonperfusion is seen in
proliferative diabetic retinopathy, in which neovascularization of the optic disc, retina, and iris may occur. Retinal
nonperfusion can also be seen in primary branch retinal vein occlusion (BRVO), and can lead to secondary retinal
neovascularization. The angiogram in this case, however, does not show capillary telangiectasia, collateral vessels, or a
pattern of involvement along the distribution of a particular vein, which are findings typically seen in BRVO. This patient's
young age, gender, ethnic background, and positive tuberculin status are characteristics commonly associated with Eales'
disease. However, there are no diagnostic tests for Eales' disease, and this diagnosis can be made only when other causes
have been excluded. The laboratory data for this patient rule against diabetes mellitus, hemoglobinopathies, sarcoidosis,
Behcet's, and systemic lupus erythematosus as underlying causes of the retinal findings. Retinal neovascularization in
Eales' disease is observed in up to 80% of patients and frequently leads to vitreous hemorrhage, and less commonly, to
retinal detachment. Laser photocoagulation of areas of peripheral retinal ischemia may produce regression of retinal
neovascularization.
Question 4 of 130
A 73-year-old man complains of increasing pain and decreasing vision OD over the last 3 days. Two weeks ago he
underwent an uncomplicated phacoemulsification, and placement of a foldable posterior chamber silicone intraocular lens
(IOL) in the same eye. On examination, his visual acuity is HM 00 and 20/30 OS. Intraocular pressures are normal. Slitlamp
examination OD reveals moderate conjunctival injection, a Seidel negative cataract wound, a 1.5-mm hypopyon in the
anterior chamber, and a fibrin plaque over the anterior surface of the IOL. A dull red reflex is present, and fundus details are
not visible. Ultrasonography shows diffuse vitreous debris, but no retinal or choroidal detachment. The patient's findings are
consistent with postoperative endophthalmitis. Based on the Endophthalmitis Vitrectomy Study (EVS) findings, what would
be the recommended initial course of treatment for this patient?
Intravenous antibiotics, immediate pars plana vitrectomy, and injection of intravitreal antibiotics
Coats' disease
Toxoplasmosis
Multifocal choroiditis
This patient is likely to demonstrate hilar lymphadenopathy and/or parenchymal infiltrates on chest x-ray.
Retinal neovascularization, which may accompany this condition, typically does not respond to systemic corticosteroids
or scatter photocoagulation.
HLA-B27 typing
Question 9 of 130
A 73-year-old man complains of increasing pain and decreasing vision OD over the last 3 days. Two weeks ago he
underwent an uncomplicated phacoemulsification, and placement of a foldable posterior chamber silicone intraocular lens
(IOL) in the same eye. On examination, his visual acuity is HM 00 and 20/30 OS. Intraocular pressures are normal. Slitlamp
examination OD reveals moderate conjunctival injection, a Seidel negative cataract wound, a 1.5-mm hypopyon in the
anterior chamber, and a fibrin plaque over the anterior surface of the IOL. A dull red reflex is present, and fundus details are
not visible. Ultrasonography shows diffuse vitreous debris, but no retinal or choroidal detachment. The patient's findings are
consistent with postoperative endophthalmitis. The patient experienced decreasing inflammation and noted improved visual
acuity for the first 2 days following treatment. On the third post treatment day, however, visual acuity decreased and a new
vitreous hemorrhage was noted. Anterior segment examination demonstrated minimal corneal epithelial edema, mild cell
and flare in the anterior chamber, a residual fibrin plaque on the anterior surface of the IOL, which is centered, and an open
posterior capsule. Ultrasonography revealed a retinal detachment. Pars plana vitrectomy, air-fluid exchange, and laser
retinopexy are planned to repair the retinal detachment, because the vitreous hemorrhage prevents an adequate view for
scleral buckling. Which of the following conditions in this patient may present the most difficulty for completing the planned
treatment?
Vitreous hemorrhage
Question 10 of 130
The ultrasonogram shown in the figure was obtained from an 18-year-old man who presented with complaints of ocular pain,
redness, mild proptosis, and blurred vision OD of 2 weeks duration. Which of the following would be least helpful in the
diagnostic workup of this patient?
Rheumatoid factor
Question 11 of 130
The ultrasonogram shown in the figure was obtained from an 18-year-old man who presented with complaints of ocular pain,
redness, mild proptosis, and blurred vision OD of 2 weeks duration. Which of the following is the most likely diagnosis in this
patient?
Idiopathic uveal effusion syndrome
Thyroid ophthalmopathy
Posterior scleritis
Please select an answer
Feedback: The ultrasound demonstrates the “T” sign that is characteristic for posterior scleritis. The ultrasound below has
an arrow added to point out the echolucent area, which is produced by retrobulbar edema sourrounding the optic nerve.
Frequently, the choroid and sclera are thickened in posterior scleritis, as shown in this patient. Occasionally, serous retinal
and choroidal detachments may also be present. Ultrasonography in idiopathic uveal effusion syndrome typically shows
thickened choroid and sclera, as well as serous choroidal and retinal detachments. Patients with this syndrome, however, do
not have retrobulbar edema, as in posterior scleritis, and typically do not present with pain or redness, which is common in
posterior scleritis. In addition, patients with idiopathic uveal effusion have a nanophthalmic eye rather than proptosis, which
may be seen in posterior scleritis. Thyroid ophthalmopathy may present with protosis, redness, and decreased vision, but
typically only demonstrates enlarged extraocular muscles on ultrasonography. VKH syndrome also does not demonstrate
retrobulbar edema on ultrasonography, but does typically show thickened choroid and serous retinal detachments.
Question 12 of 130
What does the differential diagnosis of idiopathic pars planitis include?
Ankylosing spondylitis
Rheumatoid arthritis
Lymphoma
Vogt-Koyanagi-Harada syndrome
Please select an answer
Feedback: Pars planitis is a form of intermediate uveitis featuring vitritis, pars plana snowbanks, and frequently cystoid
macular edema. It is bilateral in 90% of cases. Intraocular lymphoma can also present with bilateral vitritis, with or without a
yellowish subretinal infiltrate. Ankylosing spondylitis is associated with a recurrent acute unilateral anterior uveitis in most
cases. Rheumatoid arthritis is more commonly associated with scleritis and peripheral ulcerative keratitis. Vogt-Koyanagi-
Harada disease features bilateral granulomatous panuveitis, frequently with exudative retinal detachment, meningismus and
tinitus, and later in disease course, integument findings.
Question 13 of 130
Sterility is a side effect associated with which of the following immunomodulatory drugs?
Mycophenolate mofetil
Azathioprine
Chlorambucil
Cyclosporine A
Please select an answer
Feedback: The alkylating drugs chlorambucil and cyclophosphamide are associated with a dose-dependent risk of sterility
in both men and women. Sperm and ova banking should be discussed with patients requiring these medications.
Question 14 of 130
Which of the following parasites is associated with diffuse unilateral subacute neuroretinitis?
Taenia solium
Baylisascaris procyonis
Toxocara catis
Toxoplasma gondii
Please select an answer
Feedback: DUSN is an uncommon condition usually presenting with visual changes, photopsias, floaters, and progressing
to field loss with a 'unilateral wipeout' appearance to the fundus, similar to retinitis pigmentosum. The roundworm
Baylisascaris procyonis, which is carried by raccoons, has been implicated in this condition.
Question 15 of 130
Which form of juvenile idiopathic arthritis is most likely to be associated with anterior uveitis?
Pauciarticular
ANA-; RF+
Still's disease
Polyarticular
Please select an answer
Feedback: Between 25% and 40% of ANA+, pauciarticular onset patients with juvenile arthritis will develop uveitis. The
uveitis associated with this disorder tends to be asymptomatic, but very persistent, and is associated with very high rates of
complication.
Question 16 of 130
Which of the following is not a correct HLA association of uveitic diseases?
Question 17 of 130
Initial treatment should not include immunomodulatory therapy in addition to corticosteroids for which of the following?
Wegener's granulomatosis
Sarcoidosis
Behcet's disease
Please select an answer
Feedback: Certain uveitic conditions including Wegener's granulomatosis, mucous membrane pemphigoid, Behcet's
disease, and foveal-threatening serpiginous choroiditis need to be treated from onset with immunomodulatory drugs.
Sarcoidosis frequently responds to corticosteroid therapy alone.
Question 18 of 130
Which of the following diseases is not associated with HLA-B27 positivity?
Psoriatic arthritis
Reactive arthritis
Ankylosing spondylitis
Rheumatoid arthritis
Please select an answer
Feedback: Ankylosing spondylitis, reactive arthritis, psoriatic arthritis, inflammatory bowel disease, and undifferentiated
spondyloarthropathy are all HLA-B27 associated diseases that may all be associated with anterior uveitis. Rheumatoid
arthritis does not have a strong Class I HLA association, and is more typically associated with scleritis or keratitis than
anterior uveitis.
Question 18 of 130
Which of the following diseases is not associated with HLA-B27 positivity?
Psoriatic arthritis
Reactive arthritis
Ankylosing spondylitis
Rheumatoid arthritis
Please select an answer
Feedback: Ankylosing spondylitis, reactive arthritis, psoriatic arthritis, inflammatory bowel disease, and undifferentiated
spondyloarthropathy are all HLA-B27 associated diseases that may all be associated with anterior uveitis. Rheumatoid
arthritis does not have a strong Class I HLA association, and is more typically associated with scleritis or keratitis than
anterior uveitis.
Question 19 of 130
What is the life stage of Toxoplasma gondii associated with reactivated disease?
Tachyzoite
Cystozoite
Oocyst
Bradyzoite
Please select an answer
Feedback: The form of T. gondii responsible for uveitic disease is the tachyzoite, an asexually dividing life stage of the
parasite. Infection is typically from ingestion of oocytes, or the sexually reproducing egg form. Bradyzoites are a cyst form of
the organism which can persist latent for many years. Cystozoite is an older term for bradyzoite.
Question 20 of 130
An 84-year-old woman with a history of bilateral pseudophakia presents with a 7-day history of sudden, decreased vision in
her right eye without associated pain, redness, or photophobia. Examination reveals best corrected visual acuity of counting
fingers at 2 feet OD and 20/25 OS. Examination of the right eye shows an afferent pupillary defect, inferior keratic
precipitates, and mild flare and cell. The vitreous in the right eye contains clumps of white, cellular debris. Posterior chamber
IOLs are in position with central posterior capsular openings OU. The left eye is normal. The patient was diagnosed 4 years
ago with tuberculosis, and was treated medically for it for 1 year. Which one of the following is least likely to be helpful in
establishing a diagnosis in this patient?
Fluorescein angiography
Question 21 of 130
Which of the following drug side-effect relationships is incorrect?
Methotrexate -- hepatotoxicity
Cyclosporine A -- hypertension
Azathioprine -- sterility
Please select an answer
Feedback: Azathioprine is associated with bone marrow and hepatic toxicity. Alkylating agents are associated with sterility.
Cyclosporine A side effects include hirsutism, tremor, hypertension, and renal dysfunction. Infliximab is associated with
tuberculosis reactivation, worsening of multiple sclerosis, and possible increased risk of lymphoma. Methotrexate is
associated with hepatotoxicity and rarely pulmonary fibrosis.
Question 22 of 130
Which of the following disorders is not always bilateral?
Pars planitis
Vogt-Koyanagi-Harada disease
Birdshot chorioretinopathy
Sympathetic ophthalmia
Please select an answer
Feedback: Birdshot chorioretinopathy, VKH, and sympathetic ophthalmia are presumed autoimmune conditions that always
affect both eyes. While 90% of pars planitis cases are bilateral, a unilateral form of the disease has been described.
Question 23 of 130
What is the predominant cell type seen in the anterior chamber of a patient with phacolytic uveitis and glaucoma?
Neutrophil
T-lymphocyte
Macrophage
Eosinophil
Please select an answer
Feedback: Phacolytic glaucoma occurs when lens proteins leech from liquified cortex of a hypermature cataract into the
anterior chamber. Macrophages respond to this protein and engulf it, becoming 'lipid laden'. These cells clog the trabecular
meshwork, leading to glaucoma. Treatment requires acute corticosteroids, pressure lowering agents, and prompt removal of
the cataract.
Question 24 of 130
What would the differential diagnosis for isolated intermediate uveitis not include?
Reactive arthritis
Multiple sclerosis
Lyme disease
Please select an answer
Feedback: The differential diagnosis for isolated intermediate uveitis includes multiple sclerosis (found in 10-30% of patients
with intermediate uveitis), Lyme disease, sarcoidosis, syphilis, tuberculosis, intraocular lymphoma, and ocular ischemic
syndrome. Reactive arthritis is more typically associated with conjunctivitis and anterior uveitis.
Question 25 of 130
The 'white dot' lesions of which disease is least apparent on fluorescein angiography?
Birdshot chorioretinopathy
Serpiginous choroiditis
Question 26 of 130
Early findings in Vogt Koyanagi Harada (VKH) syndrome does not include which of the following?
Tinnitus
Vitiligo
Question 27 of 130
Which of the following uveitic conditions is most likely to present with a white and asymptomatic eye?
Wegener's granulomatosis
2 mm
15 mm
5 mm
10 mm
Please select an answer
Feedback: The PPD is read 48-72 hours after placement, and induration measured. A reading greater than 15 mm is
considered positive in all individuals. In individuals at risk, including health care workers or potential contacts with active
cases, 10 mm is considered positive. In individuals with AIDS or positive chest X-ray findings, 5 mm is considered positive.
A 2 mm PPD would be considered negative.
Question 29 of 130
What are Dalen-Fuchs nodules associated with?
Eales disease
Sympathetic ophthalmia
Ocular sarcoidosis
Behcet disease
Please select an answer
Feedback: Dalen-Fuchs nodules are small, yellow-white excrescences located between Bruchs' membrane and the retinal
pigment epithelium. They are associated with sympathetic ophthalmia and Vogt-Koyanagi-Harada syndrome.
Question 30 of 130
The fluocinolone acetonide implant releases therapeutic levels of corticosteroids to the vitreous cavity for approximately how
many days?
500
5000
1000
100
Please select an answer
Feedback: The fluocinolone acetonide implant (Retisert) releases therapeutic levels of drug for approximately 1000 days
(~3 years). Reactivation rates for intermediate and posterior uveitis are much lower in treated eyes than in untreated
controls. However, ~90% of phakic patients will develop cataract with this device and ~40-50% will require glaucoma
surgery.
Question 31 of 130
Which of the following tests is the most sensitive mode of work-up for a patient with the presentation associated with chronic
epistaxis and sinusitis in the attached images?
Serology
Scleral culture
Scleral biopsy
Question 32 of 130
Which of the following is a preferred method of treatment in a patient with primary central nervous system/intraocular
lymphoma?
Systemic chemotherapy
Local radiopathy
Please select an answer
Feedback: Intraocular lymphoma represents a form of primary central nervous system lymphoma. Treatment must be
directed at both ocular lesion and potential CNS lesions. Ocular modalities include radiotherapy and intravitreal
methotrexate. Systemic treatments include chemotherapy and intrathecal methotrexate
Question 33 of 130
Which of the following patients is most likely to have scleritis?
A 40-year-old male with red eye and throbbing eye pain with weight loss and non-healing skin lesions
A 73-year-old male with red eye and decreased vision with ipsilateral headaches and jaw pain
Please select an answer
Feedback: Scleritis is often associated with systemic vasculities, including polyarteritis nodosum, Wegener's
granulomatosis, Churg-Strauss syndrome, and systemic lupus erythematosis. Weight loss and skin lesions would be
consistent with a systemic vasculitis.
Question 34 of 130
Which of the following statements best describes the epidemiology of scleritis associated with collagen vascular disease?
Question 35 of 130
Most patients presenting with acute anterior nongranulomatous anterior uveitis will require treatment with which of the
following?
Oral corticosteroids
Question 36 of 130
Which of the following patients is most likely to have primary central nervous system/intraocular lymphoma?
A 65-year-old female with dense vitritis, subretinal infiltrates, and mental confusion
Please select an answer
Feedback: Subretinal infiltrates and mental confusion are typical for primary central nervous system/intraocular lymphoma.
The patient's age is also compatible.
Question 37 of 130
Which one of the following tests is most important for patients with either intermediate or posterior uveitis, but without
keratitis or scleritis?
HLA-B27
Please select an answer
Feedback: It is always important to rule out syphilis, a treatable disease using anti-treponemal specific testing. HLA-B27 is
rarely associated with posterior uveitis without a significant anterior uveitis, but is most often associated with recurrent
unilateral acute anterior uveitis. In any case, a positive HLA-B27 test does not make a specific diagnosis, as almost 10% of
the Caucasian population is HLA-B27 positive, so it would be little help here. Similarly, anti-nuclear antibodies can be found
in normal individuals. Neither rheumatoid arthritis (RA) nor systemic lupus erythematosis (SLE) is commonly associated with
uveitis without scleritis or retinal vasculitis. If either are suspected, a good history and physical exam are more important
than these serologies.
Question 38 of 130
Which of the following statements best describes the demographics of acute posterior multifocal placoid epitheliopathy?
Question 39 of 130
Which of the following is a complication of punctate inner choroidopathy?
Choroidal neovascularization
Optic atrophy
Retinal detachment
Please select an answer
Feedback: Up to 40% of patients with punctate inner choroidopathy (PIC) will develop choroidal neovascularization (CNV).
Inflammation in PIC is limited to the inner choroid, the vitreous is generally without inflammation, and other complications are
very rare with this condition.
Question 40 of 130
Which of the following clinical features are more common in scleritis than episcleritis?
Question 41 of 130
Mycophenolate mofetil is not appropriate for treatment of which of the following conditions?
Birdshot retinochoroidopathy
Sympathetic ophthalmia
Question 42 of 130
Which of the following statements best describes the typical fundus findings of serpiginous choroiditis?
Question 44 of 130
A 5-year-old patient with juvenile chronic arthritis and chronic anterior uveitis was started on methotrexate and achieved
control of the ocular inflammation for the first time in 6 months of therapy. However, the child now complains of abdominal
pains and refuses to take the medicine. Which approach would most likely succeed in this setting?
Administer the methotrexate pills at bedtime, so the child can sleep through the period of discomfort
Question 45 of 130
Which of the following is the most appropriate treatment for a 30-year-old man with sinus inflammation, bilateral scleritis,
mild anterior uveitis, and a positive anti-neutrophilic cytoplasmic antibodies (c-ANCA) test?
Question 46 of 130
Which of the following is a recommended treatment for serpiginous choroiditis involving the macula?
Oral corticosteroids
Topical corticosteroids
Acyclovir
Alkylating agents
Please select an answer
Feedback: Vision threatening macular serpiginous choroiditis requires prompt institution of strong immunomodulatory
therapy. Success has been reported for alkylating agents and for use of transplant-dose therapy with cyclosporine A,
azathioprine, and prednisone. Corticosteroids alone are often insufficient to treat this condition. While there has been some
discussion suggesting a herpetic genesis for serpiginous choroiditis, definitive evidence has not been demonstrated, and
acyclovir should be used adjunctively if at all. Topical corticosteroids have no effect on this condition.
Question 47 of 130
A 25-year-old, Brazilian man presents with a history of decreased vision in his left eye for 1 week. Visual acuity is 20/70, and
moderate vitreitis is present. On dilated examination, a pigmented scar in the posterior pole with adjacent yellow white
chorioretinitis is present. What is the most appropriate treatment?
Amphotericin B
IV acyclovir
Oral corticosteroids
Please select an answer
Feedback: This patient likely has ocular toxoplasmosis. While most ocular toxoplasmosis is thought to be reactivation, and
will be accompanied by a chorioretinal scar, recent work has suggested that de novo infection can also be associated with
uveitis, particularly in endemic areas, such as southern Brazil. Appropriate treatments for ocular toxoplasmosis include
combination pyrimethamine, sulfadiazene, folinic acid; trimethoprim-sulfamethoxazole; azithromycin; and clindamycin (or
combinations thereof). Amphotericin B is an antifungal agent; IV acyclovir is more appropriate for acute retinal necrosis due
to herpes virus; and oral corticosteroids without antibiotic use are contraindicated with active ocular toxoplasmosis.
Question 48 of 130
The daily dosage of cyclosporine should be adjusted based on which of the following laboratory tests?
Hematocrit
Glucose
Creatinine
Platelet count
Please select an answer
Feedback: Cyclosporine A is potentially nephrotoxic. Blood pressure and renal function should be monitored every 4-6
weeks in all patients on this medication. Cyclosporine does not have direct effects on hematocrit, platelet count, or blood
glucose.
Question 49 of 130
A 39-year-old man with AIDS and cytomegalovirus (CMV) retinitis in 1 eye undergoes successful placement of an intravitreal
ganciclovir implant without systemic anti-CMV therapy. He declines highly active antiretroviral therapy. After 1 month, the
retinitis is inactive. Nine months after surgery, the retinitis is active, and the patient's CD4+ count is 20 cells/µL. He feels well
in general, and the opposite eye shows no retinitis. What is the most appropriate approach at this time?
Question 50 of 130
In which of the disorders below is indocyanine green (ICG) angiography most useful?
Pars planitis
Posterior scleritis
Serpiginous choroiditis
Please select an answer
Feedback: ICG angiography is occasionally helpful in making the diagnosis of an individual with posterior uveitis. It can be
used, for example, in distinguishing macular serpiginous choroiditis from persistent placoid maculopathy. The former will
show resolution of the macular hypofluorescence, while the latter will show persistent hypofluorescence. That said, the utility
of ICG angiography is generally less than fluorescein angiography, as it does not show aspects of retinal circulation or
disease.
Question 51 of 130
What infectious agent has been associated with Fuchs heterochromic iridocyclitis?
Syphilis
Rubella
Herpes simplex
Herpes zoster
Please select an answer
Feedback: Both epidemiologic and polymerase chain reaction studies suggest rubella is associated with Fuchs
heterochromic iridocyclitis. This is problematic, because we do not have good agents to treat rubella virus infections.
Question 52 of 130
A 28-year-old Hispanic woman presents with three days of headache and loss of vision in both eyes. She was admitted for
suspicion of meningitis, although she has no fever. There was a mild lymphocytic response noted in her cerebrospinal fluid;
stains for microbes were negative, and cultures are negative to date. On examination she has a moderate bilateral anterior
uveitis and vitritis with serous retinal detachments. There is no retinal necrosis. What is the most appropriate therapy?
Intravitreal acyclovir
Systemic corticosteroids
Cyclophospahmide
Question 53 of 130
A 14-year-old girl complains of a sudden onset of bilateral eye pain and photophobia. On ocular examination she has
bilateral anterior uveitis. She has had a fever for two weeks, modest flank and abdominal pain, and weight loss of 10
pounds. Laboratory testing reveals an elevated erythrocyte sedimentation rate at 80 mm/hour, mild elevation of the serum
creatinine, and mild proteinuria. What is the most likely diagnosis?
Lyme disease
Toxocara uveitis
Toxoplasmosis
Please select an answer
Feedback: This case illustrates many of the common features of this rare disease. In fact, there is evidence that the
diagnosis is often missed, and that Tubulointersitital nephritis and uveitis may not be as rare as we have thought in the past.
Toxacara uveitis is not bilateral and not usually associated with systemic disease. Lyme disease can be considered, but the
sudden onset of illness without sings (such as a typical rash), or history consistent with lyme disease (or the more typical
intermediate uveitis), makes it less likely. Toxoplasmosis does not cause a bilateral anterior uveitis, and systemic
toxoplasmosis would be very uncommon in this age group.
Question 54 of 130
A 35-year-old man has decreased vision, pain, redness, and photophobia in the left eye of three days duration. He is
otherwise healthy, without known systemic disease. On examination the right eye is without inflammation. Examination of
the left eye reveals 3+ anterior chamber cells and 2-3+ vitreous cells, and haze with 5 clock hours of retinitis anterior to the
equator. He is HIV negative. What is the most likely diagnosis?
Toxoplasmosis
Toxocara
Sarcoidosis
Please select an answer
Feedback: A rapid onset of symptomatic retinitis anterior to the equator in a non-immunosuppressed patient best fits the
description of viral acute retinal necrosis. Toxocara would be an unusual cause or retinal necrosis, especially in this age
group. Sarcoidosis is not the differential diagnosis, but is less likely with this picture. A bilateral granulomatous pan uveitis,
not quite as rapid in its course, would be much more typical, although retinal necrosis is possible. Toxoplasmosis is unlikely
to result in such a large area of retinal necrosis unless there is immunosuppression, including corticosteroid injection.
Question 55 of 130
What is the mechanism of action of infliximab?
Corticosteroid
Alkylating agent
Antimetabolite
Question 56 of 130
What infectious agent(s) has been associated with corneal endothelial and subendothelial changes, leading to corneal
edema in an eye with recurrent unilateral anterior uveitis, in a patient who is not immunosuppressed?
Toxoplasmosis
Bartonella
Rubella
Please select an answer
Feedback: Cytomegallovirus anterior uveitis has been shown by polymerase chain reaction techniques to be associated
with anterior uveitis, often with endothelial changes and corneal edema that can recur in corneal grafts, in non-
immunosuppressed patients. Treatment is problematic, because valganciclovir is much more toxic than acyclovir or other
agents that are effective for herpes zoster or herpes simplex. Rubella has been associated with Fuchs heterochromic
iridocyclitis. Bartonella and toxoplasmosis ocular disease rarely, if ever, would present with an isolated unilateral anterior
uveitis leading to corneal edema.
Question 57 of 130
A patient is being evaluated by a rheumatologist for the possibility of sarcoidosis. While the patient has no ocular complaints
and has a normal eye exam, the rheumatologist requests that a conjunctival biopsy be performed to help confirm the
diagnosis of sarcoidosis. Which of the following is the most appropriate response to the rheumatologist?
"We should rely on laboratory testing, such as angiotensin converting enzyme or gallium scan, instead of biopsy."
"Blind conjunctival biopsies without a visible lesion will have a low diagnostic yield."
Please select an answer
Feedback: Sarcoidosis is a tissue diagnosis, and efforts should be made to identify involved tissues for biopsy. If
conjunctival granulomas are observed, they may be biopsied with high diagnostic yield. In the absence of visible conjunctival
lesions, however, biopsy yields are less than 10%. Higher yield may be obtained by chest CT scanning followed by
mediastinoscopy or bronchoscopy. However, such invasive diagnostic tests should be considered only when making the
sarcoidosis diagnosis has systemic treatment implications for the patient.
Question 58 of 130
Symptoms of serpiginous choroidopathy include which of the following?
Scotoma
Pain
Redness
Photophobia
Please select an answer
Feedback: Serpiginous choroiditis causes acute inflammation of the choroid and choriorcapillaris, with subsequent loss of
retinal pigment epithelium and photoreceptors. The resulting retinal dysfunction manifests as scotoma. Pain, photophobia,
and redness are typically associated with anterior uveitis.
Question 59 of 130
A 64-year-old Caucasian woman presents with a history of floaters and mildly blurred vision for two weeks in her left eye. On
examination of the right eye there is no inflammation. The left eye has mild anterior uveitis, moderate vitritis, and a localized
area of retinitis of about one half disc diameter in size nasal to the optic nerve head, but posterior to the equator of the
globe. The optic nerve head is not involved. She is otherwise healthy with no known systemic disease and is on no
medications. What is the most likely diagnosis?
Behcets disease
Toxoplasmosis
Toxocara
Please select an answer
Feedback: Toxoplasmosis is the most common cause of unilateral retinitis in this population. Toxocara would be unlikely in
this age group. Acute retinal necrosis is most often posterior to the equator, and after two weeks would have likely enlarged
ion a circumfrential fashion. Behcets disease is unlikely in a middle aged Caucasian woman without other findings and is
certainly less likely than toxoplasmosis.
Question 60 of 130
A 34-year-old man has a localized area of scleral thinning 5 mm nasal to the limbus. The surrounding episcleral and
conjunctival tissue is not injected. The patient had eye surgery four years before presentation. He is healthy without any
systemic inflammatory or infectious disease. What is the most likely surgery he had that could result in this finding?
Trabeculectomy
Laser retinopexy
LASIK
Please select an answer
Feedback: Necrotizing scleritis certainly raises concerns about systemic disease, but one must consider other causes
(foreign bodies, infection, previous mitomycin or radiation). The nasal position would make a trabeculectomy with mitomycin
unlikely, but is a typical location for pterygia.
Question 61 of 130
A patient with a bilateral anterior and intermediate uveitis is suspected of having sarcoidosis. There are no conjunctival or lid
granulomata. A chest x-ray shows no abnormalities, and serum angiotensin converting enzyme is normal. Which one of the
following is the most appropriate examination for pursuing the diagnosis of sarcoidosis?
HLA-B27 status
Question 62 of 130
Which one of the following is most important in diagnosing Behcet's disease?
HLA-B51 status
Question 63 of 130
A 47-year-old woman with birdshot chorioretinopathy has 20/40 Snellen visual acuity. She has received no treatment for this
disease. What is the most likely case of decreased Snellen visual acuity?
Cataract
Retinal vasculitis
Glaucoma
Please select an answer
Feedback: The most common cause of decreased visual acuity in birdshot chorioretinopathy is cystoid macular edema.
Glaucoma or cataract formation is unusual in birdshot chorioretinopathy except secondary to corticosteroids, because there
is little anterior uveitis. Retinal vasculitis is common in birdshot chorioretinopathy, but is not commonly associated in itself
with decreased visual acuity.
Question 64 of 130
A 23-year-old man presents complainig of floaters in his left eye for 2 weeks. The visual acuity is 20/20 in both eyes. There
is a mild (1+) anterior chamber and vitreous reaction, with a small focus of retinitis (less than 1/2 disc diameter) adjacent to a
chorio-retinal scar, anterior to the vascular arcades in the left eye. He is HIV negative. He is allergic to sulfa medication.
What is the most appropriate next step?
Question 65 of 130
Retinal vasculitis affecting only the veins is most likely to be associated with which of the following?
Behcet disease
Ocular sarcoidosis
Polyarteritis nodosa
Please select an answer
Feedback: A pure retinal periphlebitis is most likely to be associated with ocular sarcoidosis. The form of this inflammation
has been called 'candlewax drippings' or Taches de Bougie. Polyarteritis nodosa is typically associated with arteritis in the
eye. Systemic lupus erythematosis and Behcets disease may present with mixed arterial and venous inflammation.
Question 66 of 130
Which of the following approaches is most appropriate for cataract surgery in a patient with Fuch's heterochromic iridocyclitis
without glaucoma?
Start acyclovir, because Fuch's heterochromic iridocyclitis has been associated with rubella virus
Start methotrexate with a goal of no more than rare cell before surgery
Perform a lensectomy and vitrectomy, and leave the eye aphakic to prevent ciliary body membranes and hypotony
Please select an answer
Feedback: Eyes with Fuch's heterochromic iridocyclitis tend to do well with standard cataract surgery techniques and
intraocular lens (IOL) implant placement. Some uveitis specialists will treat briefly before surgery with topical and even oral
corticosteroids, but systemic immunomodulatory therapy such as one with methotrexate is not usually pursued as one would
pursue for chronic anterior uveitis associated with such diseases as sarcoidosis, VKH disease, or juvenile rheumatoid
arthritis. FHI has been associated with Rubella virus, but acyclovir is not an effective treatment.
Question 67 of 130
A 27-year-old man presents with a 2-day history of redness, pain, and photophobia in the left eye. The right eye is normal.
His Snellen visual acuity is 20/20 in the right eye and 20/25 in the left. In the left, there is a mild ciliary flush and 3+ cells in
the anterior chamber with no keratic precipitates or posterior synechiae. There is no posterior segment inflammation in either
eye. What is the most appropriate treatment?
Topical prednisolone acetate 1% 4 times/day, and posterior subtenon injection of 40 mg triamcinolone acetonide
Topical prednisolone acetate 1%, 4 times/day and oral prednisone 40 mg with a rapid taper
Please select an answer
Feedback: Although this patient may do quite well on a lower dose of topical corticosteroids, it is too early to tell, and so it is
better to treat aggressively with topical steroids as if a hypopyon and fibrin may develop. On the other hand, the patient most
likely will not need oral or injected corticosteroids, so there is no reason at this point to increase the risk of treatment by
using those modalities. He should be closely followed and the treatment tailored according to how his eye responds.
Question 68 of 130
Which one of the following tests is most likely to give a definitive diagnosis in a immunocompetent patient with a rapidly
progressing peripheral retinal necrosis in one eye?
Herpes serologies
Toxoplasma serologies
HLA-B27 status
Please select an answer
Feedback: The age of the patient and presentation are most consistent with toxocara. A complete blood count could show
abnormalities in various forms of uveitis, and possibly eosinophilia in toxocara, but other than in leukemia or systemic
lymphoma, it is not likely to lead to a specific diagnosis. This presentation is not typical of HLA-B27 disease and is postiive in
5-10% of the normal population, so even if the child is HLA-B27 positive, it would not be helpful. Similarly, herpes titers are
postivie in the majority of the population and a granuloma is not typical of herpetic disease.
Question 70 of 130
A patient with Vogt-Koyanagi-Harada disease was treated with high-dose oral prednisone and had resolution of her serous
retinal detachments. The dose has been gradually decreased, and 4 months into therapy, she is on prednisone 20 mg daily.
She is on topical prednislolone acetate 1% QID and her visual acuity is 20/30 both eyes. There are 2+ cells in the anterior
chamber of both eyes with no vitritis, cystoid macular edema, or subretinal fluid. What is the most appropriate next step?
Blood cultures
Multifocal choroiditis
Birdshot retinochoroidopathy
Propionibacterium may cause a recurrent granulomatous iridocyclitis that may not develop until months after cataract
surgery.
Nd:YAG laser capsulotomy may cause a flare-up of the intraocular inflammation in Propionibacterium endophthalmitis.
Please select an answer
Feedback: Propionibacterium acnes is a Gram-positive, anaerobic, pleomorphic bacillus that is commonly present in the
normal conjunctival flora. It has been recognized as a cause of chronic, recurrent, often granulomatous inflammation
following cataract extraction. The inflammation typically associated with Propionibacterium acnes may not develop for weeks
to months after uncomplicated cataract surgery. The organism may be sequestered in the capsular bag and a characteristic
capsular plaque may be visible. Flare-up of intraocular inflammation has been reported following Nd:YAG laser
capsulotomy, which may expose or release more organisms. Inflammation from Propionibacterium acnes typically shows a
transient response to corticosteroids, but intraocular administration of antibiotic with or without vitrectomy, capsulectomy,
and/or IOL removal or exchange are the preferred treatments for Propionibacterium acnes endophthalmitis.
Question 74 of 130
What is the most common cause of decreased visual acuity from the inflammation in idiopathic intermediate uveitis?
Retinal detachment
Glaucoma
Vitreous hemorrhage
Question 75 of 130
A 28-year-old man with acquired immunodeficiency syndrome (AIDS) presents with the right fundus shown in the figure.
Which of the following statements regarding the patient's condition is most accurate?
The lesions may represent either cotton-wool spots or early CMV retinitis. Close observation of the patient for change
in these lesions, with documentation of the fundus by photographs, is indicated.
The lesions most likely are cotton-wool spots, which are a manifestation of human immunodeficiency virus (HIV)
related noninfectious retinal vasculopathy, and do not require close followup.
If the patient is without symptoms, and no retinal hemorrhage is associated with these lesions, they do not represent
CMV retinitis.
The lesions most likely represent early CMV retinitis if the patient's CD4 lymphocyte count is less than 50 cells per
cubic mm.
Please select an answer
Feedback: These white retinal lesions may represent either cotton-wool spots, a manifestation of HIV-related noninfectious
retinal vasculopathy, or the earliest manifestation of CMV retinitis. Both the retinal vasculopathy associated with HIV
infections and CMV retinitis are typically late manifestations of AIDS. CMV retinitis, in particular, is more common when CD4
lymphocyte counts are reduced to 50cells/cubic mm or less. CMV retinitis often may present without symptoms, and
although the retinal lesions are characteristically areas of hemorrhagic retinal necrosis, atypical presentations of CMV
retinitis may occur. Therefore, the safest course in this case is to document the fundus appearance with photographs, and
observe closely for any enlargement or change in these lesions, which would indicate CMV retinitis. Close observation of the
patient for change in these lesions, with documentation of the fundus by photographs, is indicated.
Question 76 of 130
A 30-year-old woman complains of blurred central vision in the right eye for the past 3 days. Multiple chorioretinal lesions
are noted in both eyes. Which of the following findings is most consistent with a diagnosis of acute posterior multifocal
placoid pigment epitheliopathy (APMPPE)?
High-dose systemic corticosteroids are the initial treatment of choice in sympathetic ophthalmia.
Sympathetic ophthalmia may be differentiated from Vogt-Koyanagi-Harada syndrome by the absence of vitiligo and
alopecia in the former.
Question 78 of 130
Choroidal hemangiomas are not often associated with which of the following findings?
multiple sclerosis
Behcets disease
B-cell lymphoma
toxoplasmosis
Please select an answer
Feedback: This symptom is Lehrmitte’s sign, which is found in patients with multiple sclerosis. The two most common
underlying entities associated with intermediate uveitis are multiple sclerosis and sarcoidosis. The other entities can be
considered, but are unlikely in this patient.
Question 80 of 130
A 27-year-old woman in good health presents with a 6-week history of floaters OU, and a 3-week history of blurred visual
acuity OD. The fundus findings in the right eye are shown in the figures (posterior pole, A; inferior temporal periphery, B).
Examination of the left eye was significant for anterior vitreous cells, and a small pars plana snowbank. What is the most
likely diagnosis in this patient?
Pars planitis with peripheral retinal angioma
Coats' retinopathy
Flucinolone implant
Intraveous cyclophosphamide
Topical steroids
Please select an answer
Feedback: Oral nonsteroidal anti-inflammatory drugs are effective first line therapy for minimally symptomatic scleritis. If
these agents fail to suppress the inflammation, oral corticosteroids may be considered. Subtenons triamcinolone (STK) can
be used to treat non-necrotic scleritis, however, because of the risk of inducing a scleral melt, STK use is controversial.
Topical corticosteroids have littel effect on scleritis. A flucinolone implant is effective for posterior uveitis, but is not useful for
scleritis. Cyclosporin can be administered orally or topically, but not given intravenously for ocular disease.
Question 82 of 130
Which of the following patients would have the highest likelihood of developing posterior capsular opacification following
uncomplicated cataract extraction with posterior chamber lens implantation?
Question 83 of 130
A 33-year-old male presents with a first episode of moderate (2+) anterior chamber cells and flare, and 3 clock hours of
posterior synechiae. Ophthalmoscopy demonstrates 2+ vitreous haze and scattered, chorioretinal lesions. How would this
uveitis be characterized?
Panuveitis
Posterior uveitis
Please select an answer
Feedback: The presence of uniform severity of inflammation of the anterior segment, vitreous, and choroid would qualify as
a panuveitis. In cases in which inflammation of one site, such as the posterior segment, result in "spill-over" inflammation in
other ocular compartments or tissues, a panuveitis may be mis-classified.
Question 84 of 130
A 34-year-old man presents with floaters and decreased vision in the left eye. He denies any extra-ocular disorder. His right
eye examination is normal. The left eye visual acuity measures 20/40, he has 1-2+ vitreous cells, and 3 areas of
circumferentially oriented retinal whitening in the midretinal periphery. What is the most likely diagnosis?
Lymphoma
Ocular toxoplasmosis
Please select an answer
Feedback: The most likely diagnosis, given this constellation of findings in an otherwise healthy patient, is acute retinal
necrosis (ARN). All of the alternative diagnoses given can mimic some features of ARN. CMV develops in
immunosuppressed hosts, such as those with AIDS or prior organ or bone-marrow transplant. In the immunosuppressed
host, anterior chamber and vitreous cellular reaction is minimal or absent. Toxoplasmosis presents with a single region of
chorioretinitis, unless it complicates an immunosuppressed host. Lymphoma is rare in this age group.
Question 85 of 130
What is the most common ocular complication of rheumatoid arthritis?
Episcleritis
Keratoconjunctivitis sicca
Scleritis
Oral acyclovir
Intravenous acyclovir
Intravenous ganciclovir
Oral valacyclovir
Please select an answer
Feedback: Intravenous acyclovir reaches near immediate virostatic levels and has excellent ocular penetration. Many
uveitis specialists now also advise adjunctive intravitreal antiviral (foscarnet or ganciclovir). Oral valacyclovir achieves high
serum levels of drug, but the delay to achieve therapeutic levels for macular or optic nerve threatening ARN may be
unacceptable. Oral acyclovir is irregularly absorbed, but should not be relied upon for immenently threatening disease.
Ganciclovir has activity against herpes simplex and zoster, but is not a first line treatment.
Question 87 of 130
A 37-year-old man with prior anterior uveitis has progressively increased inflammation each time steroid eyedrops are
stopped. He had an exacerbation a week ago, and the inflammation has largely resolved on a tapering schedule of topical
corticosteroids. He has developed a posterior subcapsular cataract and posterior synechiae right eye. Because of recently
reduced vision to 20/80 he wants cataract surgery as soon as possible. What approach to cataract surgery would be
preferred?
Perform prompt cataract surgery and treat eny post-operative inflammation with aggresive topical corticosteroid
Please select an answer
Feedback: The widely accepted recommendation is to control the intraocular inflammation for at least 3 months before
performing elective surgery. Achieving suppression of uveitis may require immunosuppressives such as methotrexate, but
these typically require months to reach their maximum and stable level of effectiveness. In cases in which inflammation
cannot be suppressed to anterior chamber accelularity, perioperative corticosteroids can be considered as adjunctive
therapy, but with increased risk of perioperative uveitis exacerbation.
Question 88 of 130
What laboratory test has the highest predictive value for acute Bartonella burgdoferi infection?
Question 89 of 130
Cyclosporine is thought to reduce chronic noninfectious intraocular inflammation by reducing the activity of which cell type?
Macrophages
B-lymphocytes
Langerhans cells
T-lymphocytes
Please select an answer
Feedback: Cyclosporing primarily affects T-lymphocytes. Rituximab has an antibody to an antigen expressed on B-
lymphocytes, but may be effective in diseases in which T-lymphocytes play a role. Langerhans cells are antigen-presenting
cells in the skin and ocular surface that are involved in initiating the inflammatory response. Macrophages are involved in
execution of the inflammatory arc, but they are not directly modulated by cyclosporin
Question 90 of 130
A patient with deep ocular pain and subretinal fluid is suspected of having posterior scleritis with an exudative retinal
detachment. What finding would confirm a diagnosis of posterior scleritis?
Vitritis
Vitreous hemorrhage
What form of uveitis is most likely to follow gastroenteritis from Salmonella contaminated food or a Chlamydial urethritis?
Birdshot retinochoroiditis
Serpiginous retinochoroiditis
HLA-B27-associated acute anterior uveitis has followed bacterial infections, particularly gram negative rods (and chlamydia)
of mucosal surfaces. It is not an active infection, but most likely an immune response stimulated by the mucosal infection.
Although this is fairly well established, in most patients with HLA-B27 uveitis, no such previous infection is known to have
occurred. In fact, while the association is well established, most patients with acute anterior uveitis have no history of
infection.
In rare cases a gastrointestinal bacterial infection could lead to perforation or a para-colinic abcess; in such cases metastatic
endophthalmitis must be considered. Similarly if there is severe or recurrent gastrointestinal disease, Whipple's disease or
inflammatory bowel disease must be considered.
Although many forms of uveitis may be precipitated by infection or in fact be infectious, in most cases, a specific association
has not been proven. Diffuse unilateral subacute neuroretinitis is due to a nematode. Serpiginous and birdshot have been
thought to be precipitated by infections, possibly viral, but this has not been established, and treatment with
immunosuppressive agents appears to be effective, making active infection unlikely.
Question 92 of 130
In multifocal choroiditis and panuveitis, which clinical finding would most strongly influence you to recommendation chronic
systemic immunosuppression?
Choroidal neovascularization
Please select an answer
Feedback: Subretinal fibrosis is a very difficult and potentially blinding complication of posterior uveitis. If there is ongoing
inflammation in the setting of progressive fibrosis, then aggressive therapy must be considered, although indeed it is unclear
whether it is effective it is in all cases. It is important to determine whether choroidal neovascular membrane is present,
which may need more urgent therapy than the slow onset of most immunosuppressive therapy (including possibly anti-
VEGF therapies). It is not established whether intravitreal corticosteroids, such as a flucinolone implant, may be an
alternative once control is obtained.Both anterior uveitis and cystoid macular edema may require chronic
immunosuppressive therapy, or may be controlled by other intervention, but are not the strongest indications for such
therapy in this disease.
Question 93 of 130
A 29-year-old caucasian woman complains of new floaters in both eyes. She has been camping in the eastern United States
and does not remember any tick bites, but does recall a red rash on one leg followed by bumps on her shins. She denies
any sensory or motor neurologic symptoms. In both eyes she has vitritis without any retinal or choroidal lesions. Her chest x-
ray and serum angiotensin converting enzyme (ACE) are normal. What is the preferred working diagnosis?
Sarcoidosis
Lyme disease
Please select an answer
Feedback: Given that the patient went camping in a Lyme endemic area and had a rash and symptoms of erythema
nodosum, the diagnosis of Lyme disease is highly likely. However, ACE and chest x-ray are fair screening tests for
sarcoidosis, which lowers this the likelihood of this disorder. However, if Lyme titers are negative, additional testing for
sarcoidosis should be pursued. Demyelinating disease is unlikely. With this constallation of signs and symptoms, Lyme
disease is more likely. Systemic lupus erythematosis is unlikely to present in this manner.
Question 94 of 130
A 32-year-old man with necrotizing scleritis and a history of recent onset sinus disease presents with necrotizing scleritis. On
physical examination, there are no other systemic complaints or findings. What laboratory test is most likely to lead to a
specific diagnosis?
Question 95 of 130
Which of the following is a known side effect of cyclosporine therapy?
Renal insufficiency
Glaucoma
Cataract
Please select an answer
Feedback: Renal insufficiency and systemic hypertension are probably the most common side effects of concern with
cyclosporine. Cataract and glaucoma and central serous choroidopathy can occur from corticosteroid use by any route;
cystoid macular edema is not a complication of corticosteroids but is not known to be a side effect of cyclosporin.
Question 96 of 130
A 36-year-old African-American man presents with uveitis in his left eye. Slit lamp examination demonstrates 2+ anterior
chamber cells with 1+ flare and 2 posterior synechiae in the left eye. Numerous anterior vitreous cells also are present just
behind the lens. By ophthalmoscopy, no chorioretinal scarring is present. The right eye also has 2 posterior synechiae, but
no anterior chamber cells or flare, and there are no anterior vitreous cells or posterior segment abnormalities. How would
you classify this uveities episode?
Anterior uveitis
Panuveitis
Intermediate uveitis
Please select an answer
Feedback: Classifying the anatomic location of uveitis depends upon identifying the principal tissue affected. Usually this
corresponds with the site of greatest inflammation. The presence of "spill-over" cells in the anterior vitreous does not qualify
as intermediate uveitis, and would potentially confound formation of an appropriate differential diagnosis. Similarly, anatomic
complications such a cystoid macular edema do not change the classification. In this case the patient has clear evidence of
anterior but not intermediate or panuveitis.
Question 97 of 130
A 32-year-old Hispanic woman with prior multifocal choroiditis and panuveitis presents with a sudden onset of vision loss
OD. She has been off any therapy for 4 years. On examination, visual acuity is 20/200 OD with no anterior chamber
inflammation or vitritis. What findings would you expect on a fluorescein angiogram?
Sarcoidosis
Question 99 of 130
High dose corticosteroids have controlled the necrotizing scleritis associated with Wegener's granulomatosis in your patient.
What would be the preferred second intervention?
Continue high-dose corticosteroids (20 mg/day) for one year and then add other agents as needed.
Whipple's disease
HLA-B27-associated uveits
Behcets disease
Demyelinating disease
Please select an answer
Feedback: There is an association between intermediate uveitis and multiple sclerosis; it is not rare. One can have uveitis
and neuropathies with Behcets disease, but this would be very rare, and longstanding intermediate uveitis would be a very
atypical form of uveitis in Behcets disease. Whipple's disease might be considered as there are many manifestations, which
could include intermediate uveitis and neurologic involvement, but it is very rare and much less likely given the description of
Lehrmitte's sign in this patient. Intermediate uveitis, except secondary to severe anterior uveitis, is not usually an HLA-B27-
associated form of uveitis.
A false-positive RPR is possible in late syphilis, which uveitis is likely to be. A FTA-ABS is more likely to be positive, with
false negatives being very rare. Other treponemal absorption tests, such as the microhemagglutination-Treponema pallidum
(MHA-TP), are also useful. One problem with the FTA-ABS is false positives, especially if weakly reactive. Although treating
with penicillin may be reasonable, and severe and fatal reactions are rare, the best next step is to try to diagnose. Blood
cultures are not feasible for diagnosing syphilis. Availability of darkfield microscopy is very limited, it requires great expertise,
it can result in a false negative, and although a positive result would clinch the presence of spirochetes, it would not
necessarily confirm syphilis.
Question 102 of 130
What is the most common cause of visual loss in patients with birdshot chorioretinopathy?
Retinal detachment
Macular edema
Choroidal neovascularization
Retinal neovascularization
Please select an answer
Feedback: Cystoid macular edema is a common cause of decreased vision in birdshot chorioretinopathy. It is the most
common cause of mild and severe loss of Snellen visual acuity, although retinal atrophy late in the disease can also cause
severe visual disability. Retinal neovascularization is very rare, and unless there is vitroretinal traction or hemorrhage, rarely
causes vision loss. Choroidal neovascularization is also much less common than macular edema, but can cause severe loss
of central vision in birdshot chorioretinopathy.
Methotrexate and intensive topical corticosteroids in the perioperative cataract surgery period
Increase the prednisolone actetate 1% to every hour in the cataract surgery perioperative period
The standard is to maintain good control of the inflammation for 3 months before pursuing surgery. It is perilous to do
otherwise unless the uveitis is very mild and without sequelae, and certainly would be appropriate for this patient who has
chronic disease with recurrences whenever she decreases the topical medications to a reasonable, sustainable level.
Recurrences of uveitis or chronic active uveitis can cause great damage, and so it is important to make sure the
inflammation is well controlled on a regimen the patinet is tolerating before surgery. Inflammation after cataract surgery can
result in a devastating and presistent cystoid macular edema in uveitis patients, or membrane formation with traction on the
retina or cilliary body with damage to those tisses and complications such as a retinal detachment or hypotony. If a lens
implant is placed there can be "cocooning" of the lens implant with membranes and lens implant displacement causing
glaucoma and corneal decompensation and traction on the ciliary body leading to hypotony and even phthisis. If the
inflammation is severe or persistant precipitates on the lens implant that can be visually significant. This patient is unlikely to
maintain long-term control or take hourly drops for many months without systemic immundulatory therapy such as
methotrexate. However, methotrexate takes more than 2 weeks to get the full effect, and it is important to be certain that the
patient will tolerate the medication long-term, therefore giving it for only 2 weeks and mainataining control short-term with
topical corticosteroids is insufficent. The fluocinolone implant is not approved for anterior uveitis. Also it is a relatively low
dose of corticosteroid and would take a while to be effective; meanwhile damage can occur. Such an approach is feasible if
there is chronic cystoid macular edema and that is the indication for the implant. Indeed the anterior uveitis may respond
over time, but again, any such response will take time. The symptoms and presence of posterior synechiae, the nature of
the keratic precipitates, and the response to coriticosteroids indicate that this is not Fuchs heterochromic iricocyclitis (FHI),
which can be bilateral. This is important because FHI does not require such aggressive preoperative therapy.
Toxoplasmosis infection
Lymphoma
Please select an answer
Feedback: Intraocular infection with herpes simplex or zoster are by far the most common causes of ARN in an otherwise
healthy individual. ARN is defined as a peripheral, rapidly progressive retinal necrosis with an inflammatory response. There
can also be posterior areas of involvement, and even the optic nerve may be involved, but the peripheral lesions are most
characteristic. CMV retinitis causes retinal necrosis, but without as much inflammation, and does not progress as rapidly as
ARN. CMV retinitis is typically found in immunosuppressed patients. Toxoplasmosis causes retinitis, but unless the host is
immunosuppressed retinal involvement is not widespread and rapidly progressive. Lymphoma can present clinically like a
retinitis, but it is not as rapidly progressive as ARN and is much less common, especially in this age group, than ARN
secondary to herpes zoster or simplex. However, it is reasonable to consider these and other infections or inflammatory
processes, especially if the process progresses despite adequate antiviral therapy.
Choroidal neovascularization
Macular edema
Retinal vasculitis
Severe vitritis
Vitritis
Macular edema
Cortical cataract
Ultrabiomicroscopy (UBM)
Clinical history
Please select an answer
Feedback: Although a history of recent trauma can suggest a likelihood of phacoantigenic uveitis, the most definitive way to
confirm the diagnosis is to examine intracular fluid for the characteristic macrophages with engulfed lens material.
Additionally, cultures may be performed to rule out infection. Lens-induced uveitis has been most associated with trauma or
retained lens fragments after surgery, but also may be associated with sympathetic ophtalmia and hypermature lenses.
Further, severe uveitis with an advanced cataract may make such a clinical distinction difficult to make, and infections can
mimic lens-induced uveitis in the context of trauma or surgery and also be poorly responsive to topical corticosteroids. UBM
can be suggested by showing retained lens fragments but is not definitive and will not rule out infection. It should be noted
that if urgent surgery is planned for a disrupted lens capusle with retained lens material, whether from surgery or injury,
material for histology and culture can be obtained at the time of surgery and surgery need not be delayed.
Question 108 of 130
A patient with scleritis is found to have a high titer of antineutrophil cytoplasmic antibodies (ANCA) with a cytoplasmic
pattern (c-ANCA). What is the most likely systemic disease associated with these findings?
Polyarteritis nodosa
Wegener's granulomatosis
Rheumatoid arthritis
Jacob-Cruzfeld virus
Cytomegalovirus
Please select an answer
Feedback: Progressive multifocal leukoencepholopathy is a devastating central nervous system (CNS) infection. The virus
has been demonstrated in the ocualr tissues of AIDS patients and it has been associated with a multifocal retinitis in one
AIDS patient, but the main concern to opthtalmologists is that the CNS infection can be unmasked by immunosuppressive
agents such as mycophenolate used to treat uveitis. It is associated with the Jacob-Cruizfeld virus. Human papilloma virus
has not been reported to cause ophthalmic findings. Cytomegalovirus and varicella zoster cause ocular and CNS findings
but not progressive multifocal dystrophy.
Panuveitis
Multifocal choroiditis
Intermediate uveitis
Iridocyclitis
Please select an answer
Feedback: Uveitis is characterized by many factors including onset type, location and association with other inflammatory
conditions (disease or test result). Involvement of the vitreous and peripheral retina represents the middle portion of the
eye, compared to the anterior, posterior or entire eye (panuveitis). The term iridocyclitis refers to involvement of the iris and
ciliary body which does not apply to this patient. Multifocal choroiditis (MFC) is a descriptive term that may be associated
with a few vitreous cells but involvement of the vitreous is not a prominent feature. The choroidal inflammaiton would be
predominant with possibly secodnary retinal fondings such as "punched out" chorioretinal lesions. Panuveitis could be
considered if there were chorioretinal findings and anterior findings.
Methotrexate
Topical corticosteroids
Hypertension
Renal insufficiency
Infectious uveitis
Pediatric uveitis
Sarcoid uveitis
Idiopathic uveitis
Please select an answer
Feedback: Methotrexate is commonly used as a corticosteroid-sparing agent in noninfectious uveitis. It is contraindicated in
infectious uveitis as the infection may be worsened
Blood Pressure
Retinal atrophy
For six years, a 30-year-old woman has had poorly-controlled bilateral intermediate uveitis without any systemic symptoms.
A chest x-ray and tuberculin skin-test is normal. She has not traveled internationally. What associated disorder would
constitute a contraindication to adding a tumor necrosis factor inhibitor treatment?
Lymphoma
Tuberculosis
Sarcoidosis
Demyelinating disease
Please select an answer
Feedback:
Intermediate uveitis is one presentation of sarcoidosis. TNF inhibitors have been reported to treat sarcoidosis and not
contraindicated. Tuberculosis would be a contraindication to TNF inhibitors but the CXR and tuberculin test are negative.
Lymphoma is extraordinarily rate in a 30-year-old, unless it is metastatic, for which there is no evidence. Demyelinating
disease (multiple sclerosis) has a known strong association with intermediate uveitis, and TNF inhibitors are relatively
contraindicated as they have been associated with optic neuritis and exacerbations of multiple sclerosis.
Question 118 of 130
An immunocompetent adult has a chronic bilateral anterior uveitis. What positive blood test would suggest a specific
diagnosis and therapy?
Microhemagglutin assay (MHA-TP) or Fluorescent treponemal antibody-absorbed (FTA-ABS) are highly specific for syphilis.
The treatment would be systemic antibiotics, a specific treatment for syphilis. Any of the other tests would not be specific
for a disorder or a indicate a specific treatment. Immunosuppression is a non-specific therapy whether using corticosteroids
or immunosuppression. Serum ANA is rarely helpful in uveitis, and is more likely to be a false positive or indicate a disease
like systemic lupus which rarely if ever will cause an isolated bilateral anterior uveitis (as opposed to retinal or choroidal
vasculopathy or scleritis).a testing in of itself is not sensitive or specific enough to make a diagnosis with the possible
exception of HLA-A29 in birdshot chorioretinopathy, but that would not present with a bilateral anterior uveitis. The patient
could have sarcoidosis, but a serum angiotensin converting enzyme is at best a poor screening test with a very low
sensitivity and specificity so does not make the diagnosis.
Fluoresent ANA titers are more likely to be a false positive in this context. Bilateral chronic anterior uveitis is an unusual
presentation of systemic lupus erythematosis, and many individuals without systemic disease have positive ANA testing.
HLA testing may suggest a diagnosis, but is not specific or sensitive enough to suggest a particular therapy. For example,
the strongest HLA associations with uveitis are birdshot chorioretinopathy (HLA-A29), acute anterior uveitis and HLA-B27,
and tubulointerstitial nephritis and uveitis syndrome (which may be associated with chronic bilateral uveitis). In none of these
entities is the diagnosis made by HLA testing (although such testing may support the diagnosis) nor are therapeutic
decisions based on HLA testing. Although an angiotensin-converting enzyme may be useful, it is not highly specific or
sensitive for sarcoidosis and alone does not suggest a specific therapy. Postive antitreponemal testing suggests a specific
therapy: antibiotic treatment at doses sufficient to treat possible neurosyphilis, unless there is reason to do otherwise.
Arthritis
HLA-B51 positivity
Chorioretinal lesions
Scleritis
A virus
A bacteria
A parasite
A fungus
Please select an answer
Feedback:
Recent epidemiologic studies and studies using polymerase chain techniques have suggested viruses, particularly rubella
and cytomegalovirus are associated with Fuch's heterochromic iridocyclitis. Previous speculation of an association with
toxoplasmosis has not been supported by PCR and was based primarily on the concurrence of inactive chorioretinal scars
and serologic positivity in some patients.
Question 122 of 130
When is it diagnostically useful to obtain an antinuclear antibody (ANA) test in a patient with isolated anterior uveitis and no
skin, joint, or other systemic problems?
Never
Always
10%
95%
50%
20%
Please select an answer
Feedback: HLA-B27 positivity is found in about 8% of the Caucasian population, but in 50-65% of patients with unilateral
AAU. The diagnosis is not made by HLA testing, which has a poor predictive negativity and positivity, nor will finding that the
patient is HLA-B27 positive change management. Being HLA-B27 positive with uveitis is, however, associated with an
increased chance of spondyloarthropathy.
Question 124 of 130
A 34-year-old myopic woman has an asymmetric chronic uveitis characterized by periodic exacerbations, a mild anterior
uveitis, vitritis, and 50-200 micron atrophic (punched-out) choroidal lesions. Her chest CT scan is normal and her HLA-A29
negative. On a previous visit to the referring ophthalmologist, A fluorescein angiography (FA) of the chorioretinal lesions
showed early blockage and late staining. What is this woman's the most likely diagnosis?
Birdshot chorioretinopathy
Sarcoidosis
Although several entities may rarely present this way, the case description is most consistent with multifocal choroiditis and
panuveitis syndrome. POHS is unlikely because of the vitreous cells present. Sarcoidosis is unlikely as the the chest CT is
normal, the anterior uveitis is typically granulomatous, and some retinal vascular inflammation is present. Birdshot
chorioretinopathy is rare at this age, usually has a positive HLA-A29, and have discreet scattered lesions
Fungus
Parasite
Virus
Bacteria
Please select an answer
Feedback:
The case presented is of cytomegalovirus anterior uveitis and corneal endothelitis, but the features are similar to the
presentation of other herpes viruses. Anterior uveitis from viruses can be associated with diffuse or localized keratic
precipitates, corneal or iris change (usually atrophy rather than nodules), and increased intraocular pressure at the
onset of disease. Although metastatic infections are from bacteria or fungi are possible, they are rarer and usually
have an suggestive history or setting. Toxoplasmosis (or other parasites) as an isolated anterior uveitis would be very
rare in an immunocompetent patient, but has been reported in the setting of AIDS.
Question 126 of 130
A patient has chronic bilateral panuveitis that began several weeks after a penetrating injury to the right eye. Both eyes have
anterior chamber and vitreous cells, and macular edema despite hourly topical corticosteroid therapy and prednisone 15
mg/day. The inflammation was reduced by higher dose oral corticosteroids, but he cannot be weaned to less than 15
mg/day prednisone without an increase of inflammation. His visual acuity is 20/80 in the injured eye and 20/60 in the fellow
eye. What therapy would be preferred next?
Control the inflammation with corticosteroids, but start non-steroidal immunosuppressive therapy.
Sarcoidosis
Toxoplasmosis
P. acnes endophthalmitis
Uveitis-glaucoma-hyphema syndrome
Rarely
Chronic anterior uveitis , by definition, persists longer than 3 months. Keeping the pupil immobile is likely to facilitate
synechiae formation long-term. The resulting synechiae typically occur in the dilated position. Less potent cycloplegia
allows some pupil movement, which may break up or prevent synechiae. In contrast, for acute anterior uveitis, atropine may
be helpful as the iris often responds poorly to cycloplegia. In this setting strong dilation may break up recently formed
posterior synechiae. Atropine might be helpful long term treatment of uveitis in unusual circumstances such as uveitis-
glaucoma-hyphema (UGH) syndrome or pigmentary dispersion syndromes. The presence of macular edema or iris
atrophy/transillumination defects does not indicate the use of atropine.
3 weeks
3 months
6 months
Intravenous cidofovir
Intravenous foscarnet
Intravenous ganciclovir
Oral valganciclovir
Please select an answer
Feedback: IV Cidofovir has resulted in severe hypotony and anterior uveitis in as many as half of the patients treated for
CMV retinitis. The other medication have not been associated with either hypotony or inflammation.