Uveitis Flashcards
What are the cell types involved in granulomatous inflammation?
epithelioid and giant cells (Langhans’, foreign body, Touton)
What are the cell types involved in nongranulomatous inflammation?
plasma cells, lymphocytes
What are the three patterns of granulomatous inflammation?
Diffuse (VKH), discrete (sarcoidosis), zonal (lens-induces)
What is the most potent uveitis antigen?
Retinal S-protein
What are the 6 major etiological groups for uveitis?
Infectious, immune response, malignancy, trauma, chemical, idiopathic
Definition of endophthalmitis
infection or inflammation of the vitreous, anterior chamber, ciliary body, and choroid
Definition of panuveitis
endophthalmitis and involvement of the sclera
Most common cause of uveitis in adults? Second most common?
Idiopathic. HLA B27 associated.
Most common cause for acute, noninfectious hypopyon iritis?
HLA B27 associated iritis
Top 3 causes of anterior uveitis in children? Young adults? Older adults?
JRA, ankylosing spondylitis, psoriatic arthritis; HLA-B27 associated, sarcoidosis, syphilis; idiopathic, sarcoidosis, HZO
What percentage of the population is HLA B27 positive?
5%
What are the four diseases in HLA B27 positive individuals associated with acute, nongranulomatous anterior uveitis?
ankylosing spondylitis, Reiter’s syndrome, psoriatic arthritis, inflammatory bowel disease
What are the two leading causes of chronic, non-granulomatous anterior uveitis?
JRA, Fuchs’ heterochromic iridocyclitis
Name 6 infectious causes of infectious, granulomatous anterior uveitis.
syphilis, TB, leprosy, brucellosis, toxoplasmosis, P. acnes, fungal (Cryptococcus, Aspergillus), HIV
What are the cells that make up non-granulomatous KPs?
lymphocytes and PMNs
What are the cells that make up granulomatous KPs?
macrophages, lymphocytes, epithelioid cells, and multinucleated giant cells
Differential for diffuse KP
Fuchs’ heterochromic iridocyclitis, sarcoidosis, syphilis, keratouveitis, toxoplasmosis (rarely)
Where are Koeppe nodules located? Are they involved in granulomatous uveitis, non-granulomatous uveitis, or both?
Located at the pupillary margin; both
Where are Busacca nodules located? Are they involved in granulomatous uveitis, non-granulomatous uveitis, or both?
Anterior aspect of iris in the mid periphery; granulomatous
Where are Berlin nodules located? Are they involved in granulomatous uveitis, non-granulomatous uveitis, or both?
Anterior chamber angle; granulomatous
Differential for hypopyon (8)
HLA-B27 associated, infection (keratitis, endophthalmitis), foreign body, JRA, Behçet’s disease, VKH syndrome, malignancy (leukemia, lymphoma, retinoblastoma), toxic (rifabutin)
Differential for uveitis glaucoma (5 common, 3 rare)
Common: HSV, HZV, Posner-Schlossman syndrome, Fuchs’ heterochromic iridocyclitis, sarcoidosis; Rare: toxoplasmosis, syphilis, sympathetic ophthalmia.
Recurrent uveitis with back stiffness upon waking - leading diagnosis? Workup (labs/imaging)?
Ankylosing spondylitis; HLA B27 with lumbosacral spine CT
Granulomatous uveitis - leading diagnosis? Workup (labs/imaging)?
Rule out TB and sarcoidosis; CXR Upper body gallium scan Angiotensin-converting enzyme ([ACE]; elevated in 60% with sarcoidosis; also in Gaucher’s disease, miliary tuberculosis, silicosis) Lysozyme Serum calcium Liver function tests and bilirubin PPD
Child with recurrent/chronic iridocyclitis - leading diagnosis? Workup (labs/imaging)?
JRA: ANA, RF, HLA-B8
RETINAL VASCULITIS, RECURRENT APHTHOUS ULCERS, PRETIBIAL SKIN LESIONS - leading diagnosis? Workup (labs/imaging)?
Behçet’s disease; Skin lesion biopsy, HLA B51 and B27
PARS PLANITIS AND EPISODIC PARESTHESIAS - leading diagnosis? Workup (labs/imaging)?
Multiple sclerosis; MRI and lumbar puncture
RETINOCHOROIDITIS ADJACENT TO PIGMENTED CR SCAR - leading diagnosis? Workup (labs/imaging)?
Toxoplasmosis; Toxoplasma IgM and IgG titers
RETINAL VASCULITIS AND SINUSITIS - leading diagnosis? Workup (labs/imaging)?
Granulomatosis with polyangitis (Wegener’s granulomatosis); ANCA CXR Sinus films Urinalysis
RECURRENT UVEITIS AND DIARRHEA - leading diagnosis? Workup (labs/imaging)?
Inflammatory bowel syndrome; GI consult Endoscopy with biopsy
CHOROIDITIS, EXUDATIVE RD, EPISODIC TINNITUS - leading diagnosis? Workup (labs/imaging)?
Harada’s disease/VKH syndrome; FA Lumbar puncture Brain MRI Audiometry
ELDERLY FEMALE WITH VITRITIS W/O TRAUMA - leading diagnosis? Workup (labs/imaging)?
Intraoccular lymphoma or infection; Intravitreal tap, inject if concerned for infection
UNILATERAL IRIDOCYCLITIS, FINE WHITE KP, LIGHTER IRIS IN AFFECTED EYE - leading diagnosis? Workup (labs/imaging)?
Fuchs’ heterochromic iridocyclitis; IOP Gonioscopy (looking for fine angle vessels)
Differential for anterior uveitis with poor response to steroids (8)
Fuchs’, syphilis, toxoplasmosis, keratouveitis, Lyme disease, chronic postoperative endophthalmitis, ARN, CMV
What percentage of patients with ankylosing spondylitis are HLA B27 positive?
90%
What is the most common patient demographic for ankylosing spondylitis?
Young males
Eye findings in ankylosing spondylitis? Systemic findings?
Anterior uveitis, scleritis, episcleritis; lower back pain/stiffness worse with inactivity, aortic insufficiency
What is the classic triad of reactive arthritis?
Conjunctivitis, urethritis, arthritis
What percentage of patient with reactive arthritis are HLA B27 positive?
75%
What is the gender predilection for reactive arthritis?
male>female
What infections are associated with reactive arthritis (5)?
Chlamydia, Ureaplasma urealyticum, Yersinia, Shigella, Salmonella
What are the major eye findings in patients with reactive arthritis (3)?
mucopurulent conjunctivitis, keratitis, acute anterior uveitis
What HLA markers are associated with psoriatic arthritis?
HLA B27 and B17
Do patients with psoriasis without arthritis develop uveitis?
No
What percentage of patients with inflammatory bowel disease develop anterior uveitis?
10%
What percentage of patients with Crohn’s disease develop anterior uveitis?
3%
What are possible ocular symptoms in patients with inflammatory bowel disease?
conjunctivitis, keratoconjunctivitis sicca, episcleritis, scleritis, anterior uveitis, orbital cellulitis, optic neuritis
What are associated systemic findings in patients with inflammatory bowel disease?
arthritis, erythema nodosum, pyoderma gangrenosum, hepatitis, sclerosing cholangitis
What age group is most commonly affected by Fuchs’ Heterochromic Iridocyclitis?
Young adults
Does Fuchs’ Heterochromic Iridocyclitis affect one or both eyes?
Unilateral
What retinal finding is associated with Fuchs’ Heterochromic Iridocyclitis?
Chorioretinal scars (think toxoplasmosis)
What eye findings are consistent with Fuchs’ Heterochromic Iridocyclitis?
Diffuse, small stellate KP, minimal AC rxn, no PS, iris heterochromia w/involved iris being lighter, loss of iris crypts, fine-angle blood vessels, Koeppe nodules (30%), PSC cataracts (50%)
What is Amsler’s sign?
Bleeding into the AC when creation of paracentesis or main wound due to blood vessels in the angle. Gonioscopy can also lead to bleeding.
Differential for iris heterochromia (9)
trauma (intraocular metallic foreign body), inflammation, congenital Horner’s syndrome, iris melanoma, Waardenburg’s syndrome (iris heterochromia, telecanthus, white forelock, congenital deafness), Parry-Romberg syndrome (iris heterochromia, Horner’s syndrome, ocular motor palsies, nystagmus, facial hemiatrophy), glaucomatocyclitic crisis, medication (topical prostaglandin analogues [Xalatan, Lumigan, Travatan]), nevus of Ota
Is FHI responsive to steroids?
No
What other vision threatening disease are patients with FHI at a high risk of developing? What percentage of patients develop this debase?
Glaucoma, 60%
What ocular complications can be caused by Lyme Disease (16)?
cranial nerve palsies (CN 7 most common), orbital myositis, follicular conjunctivitis, symblepharon, episcleritis, keratitis (multiple nummular stromal infiltrates), chronic granulomatous iridocyclitis with posterior synechiae and vitreous cells, intermediate or posterior uveitis, pars planitis, chorioretinitis, exudative RD, CME, BRAO, retinal vasculitis, papilledema, pseudotumor cerebri, optic neuritis, optic atrophy
What are the two main ocular findings in Posner-Schlossman syndrome?
Anterior uveitis and elevated IOP
What is the natural course of Posner-Schlossman syndrome?
Episodes are typically self-limited, may require IOP suppressants and steroids
What are the slit lamp findings for Posner-Schlossman syndrome?
Small amount of AC cell, NO KP, elevated IOP
What type of hypersensitivity reaction is Phacoanaphylactic Endophthalmitis?
Type III
What is the underlying mechanism for Phacoanaphylactic Endophthalmitis?
Eye become sensitized to lens proteins and reacts when lens proteins are detected, leading to severe uveitis with hypotony, possible secondary open-angle glaucoma
What is the definitive treatment for Phacoanaphylactic Endophthalmitis?
Remove lens
What is the most common type of intermediate uveitis?
Pars planitis (85-90%)
Which HLA type is most associated with pars planitis?
HLA DR15
What demographic is pars planitis most commonly found in?
Young adults, females > males
What are the diagnostic criteria for pars planitis?
Diagnosis of exclusion
Ocular findings in pars planitis?
Anterior vitreitis, snow balls, snow banking, peripheral retinal phlebitis, disc hyperemia, no chorioretinitis
Differential diagnosis for pars planitis (6)?
MS, toxoplasmosis, toxocariasis, sarcoidosis, syphilis, Lyme disease
Possible ocular findings in multiple sclerosis?
Bilateral pars planitis, band keratopathy, mild AC reaction, vitreous cell, PSC cataract, ERM, CME, retinal phlebitis
What is the primary cause of vision loss in par planitis? Next most common?
CME; PSC cataract
Most common cause of posterior uveitis?
Toxoplasmosis
Differential of vitreitis w/panuveitis (6)
Sarcoidosis, VKH, Behcet’s, TB, syphilis, sympathetic ophthalmia
Differential for vitreitis in post surgical patient?
Endophthalmitis, Irvine-Gass syndrome
Differential for vitreitis with choroiditis?
acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous, birdshot, multifocal choroiditis, Toxocara, POHS
Differential for vitreitis with retinitis?
ARN, CMV, toxoplasmosis, candidiasis, cysticercosis, onchocerciasis
Which layers are involved in CMV retinitis?
All retinal layers
What is the CD4 count threshold for higher risk of CMV retinitis?
CD4 <50
Retinal findings of CMV retinitis (2)?
Brushfire: indolent, granular, yellow-white advancing edge with peripheral atrophic ‘burned out’ region Pizza-pie fundus: thick, yellow-white necrosis; hemorrhage, vascular sheathing
Treatment for CMV retinitis over the first two weeks?
Loading dose of IV antivirals with lower maintenance dose thereafter, 2-3 intravitreal injections of antiviral each week for 2-3 weeks
MOA and systemic toxicity of ganciclovir?
Virostatic; neutropenia and thrombocytopenia
MOA and systemic toxicity of foscarnet?
Virostatic; renal toxicity
Systemic toxicity of cidofovir?
Renal toxicity, uveitis (50%), hypotony
Which three viruses are the most common causes of ARN?
HSV, VZV, CMV
What HLA group is associated with 50% of ARN cases?
HLA DQw7
FA findings in ARN?
Focal areas of choroidal hypoperfusion early, late staining
Treatment for ARN?
IV acyclovir x5-10 days followed by 6 weeks of PO acyclovir; PO steroids, ASA to prevent vascular thrombosis
In what time frame does the fellow eye typically develop symptoms in ARN?
4 weeks
Which complication of ARN develops in 65-90% of individuals within the first 3 months?
RRD
What patient population is PORN typically seen in?
HIV and immunocompromised
What percentage of patients with PORN have unilateral disease on presentation? What percentage develop disease in the fellow eye?
75%; 70%
Retinal findings of PORN?
multiple discrete peripheral or central areas of retinal opacification/infiltrates (deep with very rapid progression), ‘cracked mud’ appearance after resolution; vasculitis is not prominent
What is the treatment for PORN?
Foscarnet and ganciclovir - does not respond well to antivirals.
What percentage of patients with PORN will become NLP by 4 weeks?
67%
What percentage of patients with PORN will develop an RD?
90%
What are the ocular findings of congenital rubella infection? Systemic manifestations?
Salt and pepper fundus, cataract or glaucoma (very rarely both); sensorineural hearing loss
What population is most at risk for ocular candida infections?
IV drug users, patients receiving TPN
What percentage of patients with candidemia develop fungal endophthalmitis?
10% (per Freidman)
What rental findings are associated with ocular candidiasis?
anterior uveitis, retinal hemorrhages, perivascular sheathing, chorioretinitis with fluffy white lesions (puff balls; may be joined by opaque vitreous stands [’string of pearls’]), vitreous abscess; may have subretinal abscess
What culture media would candida grow on?
Blood or Sabaraud’s agar
What is the treatment of ocular candidiasis ?
Voriconazole or amphoteracin B
What are eyelid findings in leprosy?
lagophthalmos, madarosis, blepharochalasis, nodules, trichiasis, entropion, ectropion, reduced blinking
What are lacrimal findings in leprosy?
acute and chronic dacryocystitis
What are corneal findings associated with leprosy?
anesthesia, exposure keratopathy, band keratopathy, corneal leproma, interstitial keratitis, thickened nerves, superficial stromal keratitis
What are scleral findings associated with leprosy?
episcleritis, scleritis, staphyloma, nodules
What are iris findings associated with leprosy?
miosis, iritis, synechiae, seclusio pupillae, atrophy, iris pearls, leproma
What are ciliary body findings associated with leprosy?
loss of accommodation, hypotony, phthisis
What are fundus findings associated with leprosy?
peripheral choroidal lesions, retinal vasculitis
What are the infective agents responsible for diffuse unilateral subacute neuroretinitis (DUSN)?
Due to infection with small roundworm in subretinal space: dog hookworm (Ancylostoma caninum) or racoon nematode (Baylisascaris procyonis)
What is the treatment for DUSN?
Laser the offending nematode
What is the treatment for cat scratch disease?
doxycycline 100mg BID and rifampin 300mg BID x4-6 weeks
Do living or dead microfilariae cause inflammation in Onchocerciasis?
Dead microfilariae; living cause very little inflammation.
What are the two leading causes of corneal blindness?
1 trachoma, #2 Onchocerciasis
What systemic signs/symptoms are found with Onchocerciasis?
pruritus, pigmentary changes (leopard-skin appearance), chronic inflammation, scarring, maculopapular rash, subcutaneous nodules
What is the treatment for Onchocerciasis?
ivermectin, suramin, diethylcarbamazine (DEC)
What organism can cause both gut malabsorption and uveitis/retinal vasculitis?
Tropheryma whipplei
What stain is used to definitively diagnose Whipple’s disease?
PAS
What is the treatment for Whipple’s disease? What is the treatment duration?
Bactrim for at least 1 year
What is the treatment of a P. Acnes infection?
Intravitreal vancomycin and cephalosporin (resistant to aminoglycosides), removal of some or all of the capsular bag w/wo IOL exchange.
What organism should you think about if a patient develops chronic inflammation 3-6 months following cataract surgery?
P. Acnes
What is the age group affected by APMPPE? Gender predilection? Laterality?
20-40 yo; F=M; Bilateral
What is the HLA association with APMPPE?
HLA B7, DR2
What do the lesions in APMPPE look like?
Large, geographic, gray-white shallow pigmented scars within 1–2 weeks. At the level of the RPE or choriocapillaris. May have vitreous cell or mild AC reaction.
In what percentage of patients is a flu-like prodrome seen in APMPPE?
33%
What other systemic manifestations may be associated with APMPPE?
thyroiditis, erythema nodosum, cerebral vasculitis, regional enteritis
What are the FA findings for APMPPE?
initial blockage with late hyperfluorescence; window defects in old cases
What is the prognosis for patients with APMPPE?
Good - vision typically recovers to >20/40
What is the age group affected by MEWDS? Gender predilection? Laterality?
20-50 yo; F>M; Unilateral>Bilateral
What symptoms might patients with MEWDS report?
decreased vision, peripheral scotomas, shimmering photopsias
What does the fundus look like in MEWDS?
Granular appearance with small white spots in the posterior pole at the level of the RPE
What is the visual field findings in MEWDS?
Enlarged blind spot
What are the FA findings for MEWDS?
early hyperfluorescence in wreath-like configuration; late staining of lesions and optic nerve
What ERG findings are associated with MEWDS?
Reduced A wave - due to involvement of the RPE and outer retina
What is the prognosis for MEWDS?
Vision recovers over weeks, but may remain poor depending on the amount of damage in the fovea
What is the age group affected by serpiginous choroidopathy? Gender predilection? Laterality?
40-60 yo; F=M; Bilateral
What layer of the retina is affected by serpiginous choroidopathy?
Affects inner chorioretinal pigment epithelium
What is the typical presenting symptom of serpiginous choroidopathy?
Painless vision loss over weeks/months
What are the fundus findings for serpiginous choroidopathy?
geographic scars with active lesion edges often extending from the disc; new lesions are often continuous with old lesions; may have mild AC reaction, vitritis, vascular sheathing, RPE detachment, NVD, CNV (rare)
What are the visual field findings in patient with serpiginous chroidopathy?
absolute scotomas corresponding with atrophic scars
What are the FA findings for patients with serpiginous choroidopathy?
Staining of acute lesions
What is the treatment for serpiginous chroidopathy?
PO steroids and/or immunosuppressants
What is the age group affected by birdshot choroidopathy? Gender predilection? Laterality?
After 4th decade of life; F>M; Bilateral
What is the HLA association for birdshot choriodopathy?
HLA A29
What does the fundus look like for birdshot choroidopathy?
cream-colored depigmented spots scattered throughout fundus; may have mild AC reaction, vitritis, retinal vasculitis, disc edema, optic atrophy, CME, epiretinal membrane, CNV
What are the FA findings for birdshot choroidopathy?
pronounced perifoveal capillary leakage with CME
What does the ERG show with birdshot choroidopathy?
diminished rod function
What is the age group affected by Multifocal Choroiditis with Panuveitis? Gender predilection?
20-50 yo; F>M
What does the fundus look like for Multifocal Choroiditis with Panuveitis?
multiple gray-white to yellow lesions (50–350 µm) at level of choroid or RPE; vitreous and AC cells; chronic lesions become atrophic with punched-out margins, variable amounts of pigmentation, and occasionally fibrosis
What are the FA findings for Multifocal Choroiditis with Panuveitis?
acute lesions block or fill early and stain late; older lesions behave like window defects with early hyperfluorescence and late fading
What is the treatment for Multifocal Choroiditis with Panuveitis?
Steroids
What are the complications of Multifocal Choroiditis with Panuveitis?
CNV, CME
What is the age group affected by PIC? Gender predilection?
20-40 yo; typically moderately myopic women
What are the presenting symptoms for patients with PIC?
Acute scotomas and photopsias
What are the fundus findings in PIC?
small (100–300 µm) yellow or gray inner choroidal lesions; resolve over weeks to form atrophic scars that may enlarge and become pigmented; new lesions do not appear; may have serous RDs; 40% risk of CNV; no AC or vitreous cells
What is the age group affected by Acute Retinal Pigment Epitheliitis (Krill’s Disease)? Gender predilection? Laterality?
Young adults; F=M; Unilateral
Presenting symptom for Acute retinal segment epitheliitis?
Sudden, painless decrease in vision
What are the fundus findings in patients with Acute Retinal Pigment Epitheliitis (Krill’s Disease)?
clusters of hyperpigmented spots (300–400 µm) in macula surrounded by yellow-white halos; with resolution, the spots lighten or darken, but halos remain; no vitritis
What are the FA findings for patients with Acute Retinal Pigment Epitheliitis (Krill’s Disease)?
blockage of spots with halos of hyperfluorescence
What is the prognosis/disease course of Acute Retinal Pigment Epitheliitis (Krill’s Disease)?
resolves completely in 6-12 weeks
What is the differential for frosted branch angiitis?
CMV retinitis, toxoplasmosis
What is the gender predilection affected by AZOOR? ? Laterality?
F>M; usually bilateral
What are presenting symptoms of patients with AZOOR?
rapid loss of function in 1 or more regions of visual field, photopsias
What are the fundus findings for AZOOR?
Minimal findings at first; retinal degeneration late
What are the ERG findings for patients with AZOOR?
Decrease rod and cone function
What is the gender predilection for Idiopathic Uveal Effusion Syndrome?
Typically in healthy, middle-aged men
What are the presenting symptoms for patients with Idiopathic Uveal Effusion Syndrome?
decreased vision, metamorphopsia, scotomas
What are the fundus findings in patients with Idiopathic Uveal Effusion Syndrome?
serous retinal, choroidal, and ciliary body detachments; mild vitritis, RPE changes (leopard spots), conjunctival injection; key finding is shifting subretinal fluid
What are the FA findings in patients with Idiopathic Uveal Effusion Syndrome?
no discrete leakage
What is the treatment for Idiopathic Uveal Effusion Syndrome?
consider scleral resection in nanophthalmic eyes, or quandrantic partial-thickness scleral windows - steroids and immunosuppressants are not effective.
What organism can cause a pink hypopyon?
Serratia
What are the top 4 causative organisms of post operative endophthalmitis?
S. epidermidis, S. aureus, Streptococci, Pseudomonas
What percentage of post op endophthalmitis occurs in the first week?
90%
What is the number one way to prevent acute postoperative endophthalmitis?
Treat blepharitis - 85% of responsible organisms are found on the lids/lashes
What organisms are typically implicated in delayed postoperative endophthalmitis?
Propionibacterium acnes (anaerobic gram + rod) or fungal
What infective agent is often associated specifically with endophthalmitis 2/2 penetrating trauma or open globe?
Bacillus (B. cereus)
What are the major risk factors for endogenous endophthalmitis?
IVDU, immunosuppression, indwelling venous catheters, s/p intra-abdominal surgery
What percentage of patients with Candida septicemia develop endophthalmitis?
30%
What was the aim of the EVS study?
1) To compare immediate vitrectomy vs tap and injection in the setting of acute postoperative endophthalmitis 2) Determine if IV antibiotics are necessary (ceftazidime and amikacin)
What surgical procedures were the patients in the EVS study s/p?
Cataract or secondary IOL surgery
What was the vision requirement for inclusion in the EVS study?
LP or better
What treatments were given to all patients in the EVS study?
Patients were also given subconjunctival injections of antibiotics (25 mg vancomycin, 100 mg ceftazidime, and steroid [6 mg dexamethasone phosphate]), topical fortified antibiotics (50 mg/mL vancomycin and 20 mg/mL amikacin) and steroid (prednisolone acetate), and oral steroid (prednisone 30 mg bid × 5–10 days).
Which antibiotics were injected intravitreal in the vitrectomy and tap/inject arms of the EVS study?
Vancomycin and amikacin
What were the main conclusions of the EVS study?
1) Vitrectomy if patient is LP vision or worse. 2) AC tap with intravitreal injection of antibiotics should be performed at patients with endophthalmitis with vision better than LP 3)IV antibiotics were of no benefit
What are the gender and race predilection for sarcoidosis?
F>M, AA>Caucasian (10:1)
What percentage of ocular sarcoidosis is bilateral?
70%
What are the two forms of anterior uveitis seen in sarcoidosis and how do they respond to steroids?
acute granulomatous [responds well to corticosteroids] and chronic recurrent [difficult to control with corticosteroids])
What are other ocular findings of sarcoidosis?
KP, iris nodules, lacrimal gland infiltration (25%; painless bilateral enlargement), conjunctival follicles, keratoconjunctivitis sicca, episcleritis and scleritis with nodules, choroiditis with yellow or white nodules, retinal periphlebitis with candlewax drippings (granulomas along retinal venules), pars planitis, vitritis, retinal neovascularization, optic nerve granuloma; secondary cataracts, glaucoma, and band keratopathy
What other systemic finding is most common and helpful in making the diagnosis? What imaging modality or lab test is used?
Hilar adenopathy; CXR or CT
What are skin findings associated with sarcoidosis?
erythema nodosum, skin granulomas (subcutaneous nodules)
What are some other, less common, systemic findings in sarcoidosis (7)?
Bones: arthralgias Hepatosplenomegaly Peripheral neuropathy Diabetes insipidus Hypercalcemia (vitamin D metabolism abnormality) Elevated serum gamma globulin (abnormality in immunoregulation) Parotid gland infiltration (may cause facial nerve palsy from compression)
What is seen on pathology slides of sarcoidosis biopsies?
noncaseating granulomas with Langhans’ multinucleated giant cells
What test should be ordered if there is concern for lacrimal, parotids, or pulmonary involvement of sarcoidosis?
gallium scan
What are the mainstay treatments for sarcoidosis?
steroids and immunosuppressants
What is the constellation of signs seen in Lofgren’s Syndrome?
Hilar lymphadenopathy, erythema nodosum, anterior uveitis, arthralgia
What is the constellation of signs seen in Mikulicz’s Syndrome?
Lacrimal and parotid gland swelling, sicca syndrome
What are possible etiologies underlying Mikulicz’s Syndrome?
sarcoidosis, TB, lymphoma/leukemia
What is the constellation of signs seen in Heerfordt’s Syndrome?
Fever, parotid gland enlargement, anterior uveitis, facial nerve palsy
What is the classic triad for Behçet’s Disease?
oral ulcers, genital ulcers, inflammatory eye disease
What is the most common HLA association for patients with Behçet’s Disease?
HLA B51
What is the gender predilection for Behçet’s Disease? Typical age? Ethnic group?
M>F; young adults; more common in Japan and Mediterranean countries
What is the natural history/disease course of Behçet’s Disease?
recurrent, explosive inflammatory episodes with active episodes lasting 2–4 weeks
Where is the uveitis most commonly seen in patients with Behçet’s Disease?
Posterior > Anterior
What are retinal findings seen in Behçet’s Disease?
recurrent vascular occlusions, retinal hemorrhages, exudates, CME, vitritis, traction RD, ischemic optic neuropathy
Aside from eye findings, what other systemic findings may be present in Behçet’s Disease?
oral (aphthous) ulcers, genital ulcers, skin lesions (erythema nodosum, acne-like lesions, folliculitis), arthritis (50%; especially wrists and ankles), thrombophlebitis, large vessel occlusion; GI pain, diarrhea, constipation; CNS involvement (25%; meningoencephalitis, strokes, palsies, confusional states)
What is the underlying pathology of Behçet’s Disease?
obliterative vasculitis with activation of both cellular and humoral limbs of immune system
What types of glaucoma are associated with Behçet’s Disease?
Uveitic and pupillary block glaucomas
What medication is the mainstay treatment of Behçet’s Disease?
Steroids
What medication is most effective for retinal vasculitis in patients with Behçet’s Disease?
chlorambucil
What medication helps to prevent recurrences of Behçet’s Disease?
colchicine
What differentiates VHK from Harada’s disease?
Harada’s disease only involves 1 eye
What are the ocular findings of VKH?
bilateral diffuse granulomatous panuveitis, serous RDs, disc edema
What are the systemic findings of VKH?
meningeal irritation, skin pigmentary changes, and auditory disturbance
To what is the autoimmune response targeted in VKH?
melanocytes
What is the typical age, gender, and race of patients with VKH?
30-50 yo; F>M; Asian, American Indian, Hispanic
What are the 2 HLA associations for VKH?
HLA DR4 and Dw53
Describe the 4 stages of VHK
Prodrome: headache, meningismus, seizures, bilateral decreased vision with pain, redness, photophobia Syndrome: uveitis with serous RDs Chronic stage: sunset fundus, Dalen-Fuchs nodules, perilimbal vitiligo Recurrent stage: AC reaction, pigment changes; 60% retain vision >20/30
Where are Dalen Fuchs nodules located?
Between Bruch’s and the RPE
How do you differentiate VKH from SO?
VKH involves the choriocapillaris, SO spares it
What is found on the LP of a patient with VKH?
CSF pleocytosis
What is the treatment for VKH?
Steroids, cycloplegics, immunosuppressants
What is the prognosis for VKH?
60% retain 20/30 vision or better
To what is the autoimmune response seen in SO?
melanin or melanin-associated proteins in uveal tissues
What type of hypersensitivity reaction is seen in SO?
Type 4 (delayed)
How long after penetrating trauma can SO manifest?
10 days to 50 years
What is the incidence of SO in penetrating trauma?
0.1%-0.3%
What are the ocular findings seen in SO?
Koeppe nodules, mutton fat KP, retinal edema, Dalen-Fuchs nodules; may have disc edema
What are the findings seen on FA in SO?
multiple hyperfluorescent sites of leakage
What is the treatment for SO?
Steroids, immunosuppressants
What findings can be seen with ocular syphilis?
(secondary and tertiary): panuveitis, iris papules and gummata (yellow-red nodules), chorioretinitis (salt and pepper changes), optic neuritis, optic atrophy, Argyll-Robertson pupil, ectopia lentis, interstitial keratitis
What are systemic signs of secondary syphilis?
SECONDARY: chancre, rash, lymphadenopathy, condyloma lata
What are systemic signs of tertiary syphilis?
TERTIARY: CNS, aortic aneurysm, gummas
What workup is necessary for patients with ocular syphilis?
serology (VDRL or RPR, and FTA-ABS); must rule out neurosyphilis with LP
What diseases can give you a false positive VDRL?
rheumatoid arthritis, anticardiolipin antibody, SLE
What is the mainstay treatment for syphilis?
Penicillin G
What should be used to treat patients with syphilis if they have a penicillin allergy?
doxycycline, erythromycin
What corneal findings can be seen in children with congenital syphilis?
interstitial keratitis (new vessels meet in center of cornea [salmon patch]); then atrophy (ghost vessels)
What ocular findings may be present in a n ocular tuberculosis infection?
lupus vulgaris on eyelids, phlyctenule, primary conjunctival TB, interstitial keratitis, scleritis, lacrimal gland involvement, orbital periostitis, granulomatous panuveitis, secondary glaucoma and cataract, chorioretinal plaque or nodule (tuberculoma), exudative RD, cranial nerve palsies (often due to basal meningitis)
What is the standard treatment for tuberculosis?
isoniazid, rifampin, ethambutol, pyrazinamide; systemic steroids may cause a flare-up; ethambutol and isoniazid can cause a toxic optic neuropathy
