What is bilateral diffuse uveal melanocytic proliferation?;"• Rare paraneoplastic • Melanocytic proliferation of choroidal melanocytes" How do you treat blebitis?;"• Topical antibiotics only" What are the signs of bleb-associated endophthalmitis and what is the treatment?;"• Eye pain • Poor vision • Hypopyon • Vitritis • Treatment: Intravitreal antibiotics with or without PPV" Allopurinol increases the toxicity of which immunomodulatory medication?;"• 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" Treating which blood disorder with chlorambucil results in a 13.5-times risk of developing leukemia?;"• Polycythemia rubra vera" What is the mechanism of action and side effect profile of chlorambucil?;"• 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" What is the mechanism of action and side effect profile of cyclophosphamide?;"• Alkylating agent • Side effects: 33-fold increased risk of bladder cancer, hemorrhagic cystitis, infertility" What are the imaging and lab findings that help support the diagnosis of congenital lymphocytic choriomeningitis (LCM)?;"• 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" Under what CD4 count is a patient at risk for CMV retinitis?;"• CD4 count < 50 cells/mm3" What is the mechanism of action and side effect profile of rituximab?;"• 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" Which test may give false positive results for patients with ocular leptospirosis?;"• Leptospirosis is a gram negative spirochete • Therefore, it can cause falsely positive rapid plasma reagin (RPR) or FTA-Abs" Which types of posterior uveitis do NOT present with vitritis?;"• Presumed ocular histoplasmosis syndrome • Punctate inner choroiditis • Progressive Outer Retinal Necrosis • Serpiginous choroidopathy • Subacute Sclerosing Panencephalitis" What is the mechanism, route of administration, indication, and side effect profile of cidofovir?;"• 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" Which oral antibiotics have the best vitreous penetration?;"• Fluoroquinolones" What cells are targeted initially by HIV? How does HIV affect these cells?;"● CD4 T cells ○ HIV decreases the number of CD4 cells ● Macrophages ○ HIV alters the function of macrophages" When is treatment of toxoplasmosis always indicated?;"• Congenital Toxoplasmosis • Pregnant women with acquired disease • Immunocompromised patients (HIV/AIDS, neoplastic disease or immunomodulating therapy)" What is leukotriene B4 and what is its effect?;"• Inflammatory mediator that causes lysosomal enzyme release and oxygen radical formation" What is the classic presentation, exam findings, and fluorescein angiogram (FA) pattern of punctate inner choroidopathy (PIC)?;"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 " What is the classic presentation, exam findings, and fluorescein angiogram (FA) pattern of multifocal choroiditis (MCP)?;"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 " What is the classic presentation, examination, and IVFA findings of Acute Zonal Occult Outer Retinopathy (AZOOR)?;"• 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" Where are Koeppe nodules seen?;"Koeppe nodules are iris nodules located near the pupillary margin" Where are Busacca nodules seen?;"Busacca nodules are iris nodules seen in the iris stroma" Where are Berlin nodules seen?;"Berlin nodules are iris nodules seen in the iridocorneal angle" What are the risk factors for CMV retinitis and how does the virus infect the retina?;"• 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" 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 " How does Diffuse Unilateral Subacute Neuroretinitis (DUSN) differ from Ocular Cysticercosis in relation to treatment response and lab work up?;"" 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" 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" What is the prophylactic treatment for pneumocystis jirovecii?;"• Trimethoprim/sulfamethoxazole" 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" 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" 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)" Which topical steroids produce less rise in IOP?;"• Rimexolone • Loteprednol • Fluorometholone" 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" 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" 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" 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 " 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" 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" What two main side effects should be monitored for during cyclosporine use?;"• Hypertension • Nephrotoxicity" 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" 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" 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" What HLA types are associated with Vogt-Koyanagi-Harada Syndrome?;"• HLA-DRB1 • HLA-DR4: in Japanese patients" 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" 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 " What is the mechanism of action of NSAIDs?;"Inhibition of cyclooxygenase 1 and 2 " 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" “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" 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" 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" What is the differential for hypopyon in non-endophthalmitis uveitis?;"• HLA-B27 uveitis • Behcet disease • Rifabutin (medication induced, sterile hypopyon)" 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" What is the mechanism of action of Anakinra?;"• Anakinra is a recombinant IL-1 receptor antagonist" What is the mechanism of action of Adalimumab?;"• Adalimumab is a fully human monoclonal IgG1 antibody directed against TNF-alpha" What is the mechanism of action of etanercept?;"• Etanercept is a TNF receptor blocker" 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)" 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)" 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" 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" What are the contraindications to sub-Tenon steroid injection?;"• Infectious uveitis • Necrotizing scleritis" 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" 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" 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" 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" 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" 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" 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" What are the traditional DMARDs?;"● The traditional DMARDs are: ○ Methotrexate ○ Sulfasalazine ○ Leflunomide" 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" What are the typical pathogens associated with post-traumatic endophthalmitis?;"• Bacillus • S. epidermidis • Streptococci • Fungi • S. aureus" 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" 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" 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" 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" 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" 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" 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" 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" What HLA type is associated with Tubulointerstitial Nephritis and Uveitis Syndrome (TINU)?;"HLA-DRB1*0102" 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" 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" 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" 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" 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" 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)" 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" 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" Which uveitis conditions classically benefit from early steroid-sparing immunomodulatory therapy (IMT)?;"• Behçet's • Sympathetic ophthalmia • Vogt-Koyanagi-Harada Syndrome • Necrotizing sclerouveitis" 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" 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)" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" What is the most common type of primary CNS lymphoma?;"• Non-Hodgkin B Cell Lymphoma makes up > 90% of primary CNS lymphomas" 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" 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" 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" 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" 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" 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" 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)" 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" 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" 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" What are the screening and confirmatory tests for HIV?;"• HIV screening test: Antibody detection by ELISA • HIV confirmatory test: Western blot" 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" 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" 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" 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" What cytokine is elevated in the vitreous in inflammatory uveitides?;"• IL-6 is elevated in inflammatory uveitides" Among which racial groups is VKH least common?;"• VKH is least common among Caucasians and Africans" 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" 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" 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%" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" 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" What are the predominant symptoms of intermediate uveitis?;"• Floaters and blurred vision are the predominant symptoms of intermediate uveitis" 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)" 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" 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" 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" 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" What is the definition of iridocylitis?;"• Iridocyclitis is defined as anterior chamber inflammation which spills over into the retrolental space" 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" 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" 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" 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" 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" 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" What is the differential for pars planitis/intermediate uveitis?;"• Sarcoidosis • Syphilis • Toxocariasis • Lyme Disease • Multiple Sclerosis" 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" 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)" 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)" 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" 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" 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)" What are the causes of blind spot enlargement?;"• Papilledema • Chorioretinal inflammatory conditions (i.e. MEWDS, MFC) • Acute idiopathic blind spot enlargement" 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" 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" 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" 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" 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" What are the side effects of cyclosporine?;"• Hypertension • Nephrotoxicity • Gum hyperplasia • Hirsutism" 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" 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" 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" What is the classic triad of ocular histoplasmosis?;"• Peripapillary atrophy • Multiple atrophic punched out chorioretinal scars • CNVM" What is varicella zoster sine herpete?;"• Varicella zoster sine herpete is anterior uveitis secondary to VZV without skin lesions" What is the pathology of episcleritis?;"• Pathology of episcleritis shows palisading arrangement of epithelioid histiocytes around a central core of necrotic collagen" 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" 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" What is the most common causes of bleb-associated endophthalmitis?;"• Streptococcus or Haemophilus species" 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" 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" 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)" 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" 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" 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" 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" 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" 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" What is the classic triad of Behcet disease?;"• Recurrent aphthous oral ulcers (most common) • Genital ulcers • Recurrent uveitis" 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" 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''" What is the differential diagnosis for elevated IOP and uveitis?;"• Herpetic • Toxoplasmosis • Syphilis • Sarcoidosis"