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White Dot Syndromes & Uveitis Flashcards

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1
Q

Quenching on FA

A

Birdshot

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2
Q

MEWDS

A
  • poorly understood, idiopathic inflammatory condition of the inner choroid and/or outer retina.
  • u/L in younger myopic females

Si/Sx: central vision loss, enlarged blind spot, and photopsias.

  • hallmark features = short-lived white dots usually lasting from days to a week after visual symptoms begin, permanent macular pigment stippling, macular wreath-like punctuate hyperfluorescence on fluorescein angiography [smaller hyerF dots make it up], and mild disc edema in some cases.
  • SD-OCT: non-specific but DIAGNOSTIC OF MEWDS = disruption of IS/OS junction and RPE. disruption of the usual contour of these layers without evidence of retinal edema. Present even s/p white dots have resolved.

Similar changes can be seen in AZOOR, commotio retinae, and solar retinopathy.

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3
Q

Photopsias

A

AZOOR, MEWDS, idiopathic enlarged blind spot

Photopsias = prominent feature in a young patient with central vision loss should raise suspicion for these conditions.

Other causes of photopsias associated with vision loss are often short lasting (migraine), have prominent exam findings (retinal detachment or tears), or don’t typically present in a young patient (cancer-associated retinopathy and melanoma-associated retinopathy).

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3
Q

APMPPE

A

Block early stain late

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4
Q

Birdshot

A

vitiliginous chorioretinitis

  • disease typically occurs in women
  • always has some vitritis, has retinal lesions that are more numerous in the NASAL retina
  • usually bilateral.

-peculiar FA phenomenon of “quenching” can occur in this disease whereby the dye disappears quickly from the retinal circulation

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5
Q

AZOOR

A

Acute zonal occult outer retinopathy (AZOOR) is often considered among the white dot syndromes and classically presents with acute onset of photopsias and an enlarged blind spot scotoma. Initially fundus findings are often absent but over time these patients develop retinal pigment epithelium atrophy extending off of the optic nerve and corresponding perfectly to their scotoma.

DFE: ZONAL REGIONS OF ATROPHY &HYPERPIGMENTATION (RPE clumping) along ON/temporal vascular arcades.
VF: defects match RPE atrophy region. Almost always has VF emanate from blind spot.

FA: large patches of “window defect” hyperfluorescence 2/2 increased transmission of choroidal fluorescence 2/2 loss of normal RPE overlying choroid

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6
Q

Idiopathic blind spot enlargement syndromes

A

“waste basket” - MEWDS and AZOOR
Look for fundus findings.

Normal DFE = Idiopathic blind spot enlargement syndrome

DFE w/temporal retinal whitening (days-weeks) with “granularity” of the macular RPE (permanent change) = MEWDS (MEWDS resolves usually w/in 3 mo)

AZOOR > 75% prominent photopsias. 60% of time is u/L.

Unlike MEWDS, AZOOR can involve the fellow eye in 60% of u/L cases

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7
Q

Serpiginous

A

blockage in the early frames, but early hyperfluorescence at the edges of the lesions
DDx: APMPPE (acute posterior multifocal placoid pigment epitheliopathy) since both can present with large placoid yellowish lesions at the level of the RPE. BUT APMPPE lesions resolve spontaneously over a few weeks and visual prognosis is generally good.

In select cases, treatment with immunosuppressives (e.g. mycophenylate, cyclosporine, azathioprine, and prednisone) may induce remission.

According to the BCSC, CNV only rarely occurs at the margin of an area of chorioretinal atrophy.

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8
Q

PIC (punctate inner choroidopathy)

A
  • OCT: focal loss of IS/OS photoreceptor junction and RPE at fovea
  • FA: window/staining defects 2/2 loss of these layers
  • MYOPIC females (90%) 18-40 yo
  • DFE: yellow-white chorioretinal lesions 100-300 um diameter at level of inner choroid and RPE. Found OU in 80% of cases. Progress to atrophic scars –> halo of depigmentation which often appears punched out. Subretinal neovascular membranes occur in at least 1/3 of cases.

PIC and MCP = spectrum of same disease

CNV = MCC permanent vision loss in PIC

no systemic association

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9
Q

OHS vs. MCP

A

Both have punched out lesions

However, OHS has NO VITRITIS, as opposed to 90% MCP pts with vitritis.

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10
Q

MCP (multifocal choroiditis and panuveitis)

A 
poor Px (like serpiginous.
MC in females, affects OU
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11
Q

Type I

A

Anaphylactic or atopic
mast cells and histamine

Example?
Allergic conjunctivitis 
seasonal component
Atopic conjunctivitis 
environmental triggers
less seasonal
ezcema, asthma
Anaphylaxis
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12
Q

Type II

A

Cytotoxic
Foreign or autoantigens

Example?
OCP (IgA deposits in BM)
Mooren Ulcer

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13
Q

Type III

A

Immune-complex reaction

Example?
Scleritis
Vasculitis
Phacoanaphylaxis
Stevens-Johnson syndrome
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14
Q

Type IV

A

Delayed hypersensitivity
T Cell mediated

Example?
Contact dermatitis
Phlyctenulosis
Corneal graft rejection
Sarcoid, TB
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15
Q

Type V

A

Stimulating antibody
Example?
Graves’ disease

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16
Q

Giant cells (3 types)

A

1) Langhans
Nuclei in periphery
What disease?
TB, sarcoidosis

2) Touton
mid-peripheral ring of nuceli
What disease?
JXG, chalazion, xanthelasma

3) Foreign Body
Randomly-distributed nuceli

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17
Q

Anterior scleritis

A

Female > male, bilateral (50%)
Systemic associations? (50%)
RA, SLE, PAN, Wegener, relapsing polychondritis, Reiter
Syphilis, TB, VZV, Lyme, Bartonella

3 Types?
Diffuse (40%)
Nodular (40%)
Necrotizing (15%)
Most destructive

Path?
Palisading histiocytes, multinucleated giant cells, scleral necrosis

Scleromalacia perforans?
Frequent in RA
Painless, minimal inflammation
Thin sclera with herniating uvea

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18
Q

Posterior scleritis (2%)

A

Symptoms?
proptosis, restricted eye motility

Clinical Signs?
Choroidal folds, exudative RD, papilledema, ACG from choroidal thickening, lower eyelid retraction

Diagnostic testing?
Thickened posterior sclera on U/S (“T” sign), CT, or MRI

Systemic association?
None

Treatment?
Topical or oral steroids
NO subconj or subtenon steroids (can cause scleral necrosis)
Immunosupression: MTX, cyclosporine, cyclophosphamide

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19
Q

Reiter syndrome

A

Young adult males
HLA-B27
Triggered by infection (usu. GI)
Dysentery, chlamydia, ureaplasma, shigella, salmonella, yersinia

Triad? Can’t see, can’t pee, can’t climb a tree
Conjunctivitis (+/- iritis)
Urethritis
Polyarthritis

2 additional criteria?
Keratoderma blennorrhagicum: Rash on palms and soles of feet
Balanitis: rash on distal penis

Minor criteria?
Plantar fasciitis, achilles tendinitis, nail-bed pitting, palate ulcers, tongue ulcers

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20
Q

Other seronegative dx w/ uveitis?

A

Ankylosing spondylitis
Psoriatic arthritis
Ulcerative colitis&raquo_space; Crohn’s disease

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21
Q

Uveitis:
How long after quiescent for PK?
How long after quiescent to CE?

A 
CE = 3 mo
PK = 6 mo
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22
Q

Wegener Granulomatosis

A

Systemic granulomatous vasculitis

Ocular findings?
Scleritis (25%)
Posterior scleritis with exudative RD
Recurring orbital pseudotumor
Proptosis
Retinal vasculitis, retinitis

Systemic findings?
Lung & renal involvement
Sinusitis, epistaxis
Saddle-nose deformity

Diagnosis?
cANCA (95% sensitive, 88% specific)
Tissue biopsy

Treatment?
Immunosuppression (cyclophosphamide), TMP-SMX (bactrim)
80% mortality rate if untreated

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23
Q

Juvenile Idiopathic Arthritis

A 
3 types?
Pauciarticular (2 types ?)
Type 1: early-onset
80% girls
RF-, ANA+ (60%), HLA-B27-
Chronic uveitis (30%)

Type 2: late-onset
90% boys
RF-, ANA-, HLA-B27+ (75%)
Acute uveitis (15%)

===
Polyarticular
Uveitis rare
85% girls; 40% of JRA; 75% RF-
====

Still’s disease
Uveitis rare; systemic JRA w/ high fever, rash, organomegaly
60% boys, 20% of JRA
Symptoms?
Usu. asymptomatic w/ recurrent AC inflammation

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24
JIA Rx and Px
Treatment: Steroids, immunomodulation Avoid IOL after cataract surgery! Worse prognosis if? RF-, ANA+ Pauciarticular > Polyarticular Lower limb joints > upper limb
25
Blau Syndrome
Familial juvenile systemic granulomatosis Inheritance: AD Multifocal choroiditis, vasculitic skin rash, polyarthritis, no pulmonary involvement
26
DDx of iritis + high IOP?
``` Fuchs Heterochromic Posner-Schlossman Sarcoidosis Herpetic uveitis Toxoplasmosis Syphilis ```
27
Fuchs heterochromic iridocyclitis
Young, unilateral, chronic Clinical findings? Iris stromal atrophy (heterochromia) Minimal cell / flare in AC +/- ant vit Diffuse stellate KP ``` Amsler sign? Heme during paracentesis from fine angle vessels (not NV) no PAS Cataracts (cortical) Glaucoma Infectious cause? Rubella, CMV Associated with toxoplasmosis ``` Treatment? No steroids
28
Behcet syndrome
Idiopathic occlusive vasculitis Common in? Young adults; M>F Eastern Mediterranean to Japan HLA-B5 subset B51 Triad? Iritis with hypopyon Oral aphthous ulcers Genital ulcers Other ocular findings? Panuveitis, retinitis, vasculitis, optic neuritis Patchy retinal whitening (like CWS, but parallel to NFL) Maybe quiet with hypopyon Usually bilateral Other systemic lesions? Erythema nodosum, arthritis, epididymitis, GI ulcers, pulmonary artery aneurysms, CNS vasculitis + stroke Treatment? Immunosuppression (cyclosporine & azathioprine are proven in clinical trials)
29
Sarcoidosis
Black, age 20-50, M=F Ocular involvement (50%) Granulomatous ant. uveitis (most common) Mutton-fat KPs Iris nodules? Koeppe (pupil margin; both granulomatous & non-granulomatous) Busacca (iris stroma; only granulomatous) Berlin (angle; only granulomatous) ``` Posterior uveitis Vitritis, vasculitis, CME, exudative RD Candle-wax dripping on retinal veins Choroidal granulomas Retinal lesions rare Optic nerve, poss. CNS involvement Eyelid skin nodules Lacrimal gland enlargement Conjunctival granulomas Band keratopathy Nummular keratitis ``` Systemic involvement Hilar nodules Gallium scan “Panda Sign” Path? Noncaseating granuloma Langhans cell histiocytes
30
Mikulicz syndrome?
- Dry eyes from lacrimal, salivary gland involvement
31
Heerfordt syndrome?
- Constitutional symptoms, parotid swelling, facial nerve paralysis
32
Lofgren syndrome?
- Bilateral hilar lymphadenopathy, arthropathy, fever, erythema nodosum
33
Intermediate uveitis
``` Etiology? planitis Pars = Idiopathic (90%) Sarcoid, MS, Lyme, Toxocariasis, syphilis, TB, connective tissue dx 90% chronic/recurrent Usually bilateral ``` ``` Clinical signs? Snowballs in vitreous Snowbanks on pars plana FA: peripheral venular cuffing + leakage Chronic changes: PAS, band keratopathy, CME, PSC, ERM, RD, vitreous cellular opacification, peripheral retinal NV, vitreous hemorrhage ``` Treatment? Subtenon steroids, peripheral laser, IMT
34
Toxoplasmosis
Infection with T gondii (protozoan) Acquired vs. Congenital? Acquired: ingestion of bradyzoites from raw meat or cat litter) Congenital: transmission of tachyzoites from mother’s blood Ocular findings? Vitritis/retinitis next to old scars (congenital lesions usually in macula; acquired lesions usually in periphery) Headlight in fog appearance Systemic signs in congenital toxo? Seizures, cerebral calcifications If AIDS patient, check neuroimaging (30% have brain lesions)
35
Toxoplasmosis Rx
``` Treatment? Triple therapy (x 1 yr if congenital) Pyrimethamine, sulfadiazine, folinic acid +/-prednisone Bactrim DS BID Atovaquone (attacks cyst too!) 750mg TID Clindamycin (if pregnant) Intravitreal clindamycin + dexamethasone ```
36
Presumed Ocular Histoplasmosis Syndrome Rx
``` Treatment? Steroids (PO or IVt) – no clear evidence Laser (based on MPS) for CNV Anti-VEGF for CNV Submacular surgery for CNV ``` Submacular Surgery Trial (SST): Modest benefit for CNV from POHS if BCVA <20/100
37
Presumed Ocular Histoplasmosis Syndrome Dx and demographic
Presumed infection with H. capsulatum (fungus) Geographic associations? Ohio-Mississippi River Valley San Joaquin Valley Typically age 20-50 HLA association? HLA-B7 ``` Ocular findings? Punched-out chorioretinal scars (histo spots) Peripapillary atrophy CNV (5% of patients) No vitritis ``` Chest X-ray may show? Hilar adenopathy or calcifications
38
DDx Posterior Uveitis - Infectious
o. Infectious: Bacterial endophthalmitis ``` o. Viral ARN CMV EBV Rubella Measles SSPE ``` o. Fungal POHS (NO vitritis) Candidiasis o. Protozoal: Toxoplasmosis gondii o. Helminthic Toxocariasis canis Cysticercosis DUSN
39
Venules affected in posterior uveitis
Sarcoidosis MS Birdshot Eales
40
Arterioles affected in posterior uveitis
``` Arterioles SLE PAN Toxo HSV/VZV ```
41
Immunologic Posterior Uveitis
o. Collagen vascular SLE PAN Wegener granulomatosis ``` o. Retinochoroidopathies APMPPE ARPE/Krill (NO vitritis) Birdshot MEWDS PIC (NO vitritis) Serpiginous MCP ```
43
CMV retinitis Signs and path
Occurs when CD4
43
CMV Rx
Treatment? Intravitreal ganciclovir or foscarnet Induction x 2wks: IV ganciclovir (5mg/kg BID; neutropenia) IV foscarnet (90mg/kg BID; renal failure) PO valganciclovir (900mg BID x 3wks) ``` Then lower dose & switch to PO: PO valganciclovir (900mg daily) Cidofovir (renal failure, uveitis, ciliary body shutdown; needs probenicid + IVF) ```
44
Common symptom of congenital CMV?
Hearing loss
45
Acute Retinal Necrosis (ARN)
Systemic condition? Healthy NOT immunosupressed ``` Etiology? HSV, VZV, CMV Bilateral “BARN” (20%) Ocular findings? Occlusive retinal arteriolitis Severe vitritis Yellow-white peripheral retinitis Anterior uveitis RD (75%, highest risk at 8-12wks) ``` ``` Treatment? Induction x 14 days: IV Acyclovir (800mg 5x/day) PO Famvir (500mg TID) PO Valacyclovir (1gm TID) Then lower dose, switch to PO Prophylactic laser for RD ``` Visual prognosis? poor
46
Progressive Outer Retinal Necrosis
Systemic condition? AIDS Etiology? VZV, other herpes viruses Commonly bilateral w/ rapid progression Ocular Findings? Multifocal deep, dense retinal lesions Mild vitritis Spares retinal vasculature with cracked mud appearance (unlike ARN & CMV) Treatment? IV ganciclovir Intravitreal foscarnet
47
DDx of Neuroretinitis?
``` Cat-Scratch disease DUSN Syphilis Lyme Leber idiopathic stellate neuroretinitis ```
48
Parinaud Oculoglandular Syndrome
Infection by? Bartonella henselae (most common), tularemia, lymphogranuloma venereum, TB, syphilis, sporotrichosis, etc. Usually occurs 2-3 weeks after scratch ``` Ocular findings? Unilateral Granulomatous conjunctivitis Preauricular adenopathy No leakage on FA ``` Treatment? Supportive (disease self-limited) Doxycycline +/- rifampin (if worse) Other ocular manifestation of B.henselae? Neuroretinitis Disc edema, uveitis, macular star Self-limited; spontaneous improvement
49
Diffuse Unilateral Subacute
Neuroretinitis (DUSN) ``` Helminth infection by? Toxocara canis Baylisascaris procyonis (N. America) Ancylostoma caninum (Southeast U.S.) ``` ``` Ocular findings? White dots in outer retina with vitritis and papillitis “Wiped-out” fundus late Decreased ERG late ``` Treatment? laser worm
50
Cysticercosis
Infection by? Taenia solium tapeworm Death of worm causes severe inflammation
51
West Nile Virus
Transmission by mosquito Most infection subclinical (80%), or as febrile illness (20%) Ocular findings present in 80% of cases with neurological involvement Ocular findings? Multifocal chorioretinitis with characteristic “target-like” lesions in mid-periphery Randomly distributed or in linear arays FA: early hypofluorescence and late staining Non-granulomatous anterior uveitis Conjunctivitis, subconjunctival hemorrhage Optic neuritis Treatment? None; supportive therapy & infection control Usually self-limited and symptomatic
52
DDx Multifocal CSCR
``` Hypotony Posterior scleritis Sympathetic ophthalmia VKH Lymphoma Lupus Uveal effusion syndrome ```
53
Lymphoma
Most common type? Diffuse large B-cell lymphoma Multiple deep yellowish spots, mild vitritis FA finding? Leopard skin fundus ``` Elevated intraocular levels of? IL-10 Especially in elderly Must rule out CNS lymphoma with LP Treatment: Whole-brain and ocular XRT Intrathecal MTX IV cytotoxic agents ```
54
VKH
Bilateral granulomatous panuveitis Caused by autoimmunity to melanin/melanocytes Asian, Native Americans, Hispanics, 30-50 y/o Caucasians, Africans rare Stages? Treatment? Periocular steroid injections
55
VKH Stage 1.
Stage 1. Prodromal: | Viral, CNS symptoms (HA, seizures, tinnitus)
56
VKH Stage 2.
``` Stage 2.Uveitis: Choroidal NV Optic neuropathy (disc edema) Hypotony Bilateral exudative RD, but CME rare ```
57
VKH Stage 3.
Stage 3. Convalescent: Vitiligo, alopecia, poliosis Sugiura sign (paralimbal vitiligo) Sunset glow fundus (depigmented choroid)
58
VKH Stage 4.
Stage 4. Recurrent: | chronic uveitis
59
VKH Dx
CSF? Pleocytosis U/S? Diffuse choroidal thickening HLA? DR4 Path? Dalen Fuchs nodules. Also seen in SO.
60
Sympathetic Ophthalmia
``` Bilateral granulomatous panuveitis Entire uveal tract involved Antigen is uveal pigment Sparing of choriocapillaris SO has “sympathy” for the choriocapillaris, unlike VKH ```
61
Dalen-Fuchs nodules?
Collection of inflammatory cells on inner surface of Bruch’s membrane (between RPE and Bruch’s) seen in VKH & SO
62
HLA-A29 assoc/w?
HLA-A29 | Birdshot (96% strongest assocation)
63
HLA-B27 assoc/w?
Reiter’s, Ankylosing spondylitis, psoriatic arthritis, IBD Whipple’s disease JRA Klebsiella (cross reaction)
64
HLA-B5 / B51 assoc/w?
Behcet’s disease
65
HLA-B7 assoc/w?
POHS
66
HLA-DR4 assoc/w?
VKH
67
HLA-DR15 assoc/w?
Pars planitis
68
Serpiginous/MCP/Birdshot have what in common?
``` Chronic Middle-Age Poor visual outcome Needs steroid therapy Associated with CNV ```
69
APMPEE/MEWDS/PIC have what in common?
Acute Young Good visual outcome No treatment needed
70
Serpiginous/MCP/Birdshot differentiating factors?
M=F in serpiginous and birdshot F> M in MCP
71
APMPEE/MEWDS/PIC differentiating factors?
F > M in MEWDS and PIC M = F in APMPEE Viral Prodrome/Mild Vitritis: MEWDS/APMPEE Viral Prodrome/No Vitritis: PIC
72
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
Mnemonic: Acute Prodrome, Minor People, Prognosis Excellent Occurs in young patients 1/3 have viral prodrome (days to weeks earlier) Usually mild visual loss Ocular findings? Bilateral multiple creamy placoid subretinal lesions (FA: early hypo and late stain) Mild vitritis ``` Systemic manifestations? Erythema nodosum Thyroiditis Microvascular nephropathy Cerebral vasculitis Etiology unknown, likely obstructive vasculitis with non-perfusion of terminal choroidal lobules ``` Treatment & Prognosis? No treatment, good prognosis (80% has VA>20/40) Self-limited, resolves in weeks to months
73
Serpiginous Choroiditis
Occurs in middle-age patients (age 30-60) Similar to APMPPE, but chronic & recurrent Usually asymptomatic until fovea affected Ocular findings? Bilateral grayish-yellow geographic lesion, usu. emanating from optic disc w/ snake-like appearance (FA: early hypo, late stain) Mild vitritis Risk of CNV if macula involved Treatment & Prognosis? Systemic steroids for active juxtafoveal lesions, IMT (e.g. azathioprine & cyclosporine) if recurrent Anti-VEGF or laser if CNV occurs Poor prognosis (50% has central VA loss)
74
Ampiginous Choroiditis
aka Relentless Placoid Choroidopathy Ocular findings? Bilateral multifocal lesions similar to APMPPE, but progresses relentlessly over months Treatment & Prognosis? Steroid & IMT (e.g MTX, Cellcept)
75
Multiple Evanescent White Dot Syndrome
Occurs often in young women (age 20-45) 1/3 have viral prodrome (days to weeks earlier) Symptoms? Blurred vision with shimmering photopsias Enlarged blind spot Ocular findings? Unilateral white dots at level of RPE (FA: early hyper, wreath-like late stain) Mild vitritis Decreased A-wave on ERG Treatment & Prognosis? No treatment; good prognosis Self-limited (self resolve in 3-10 weeks)
76
DDx enlarged blind spot?
Disc edema ONH drusen MEWDS IEBSS
77
Punctate Inner Choroidopathy
Mainly in young myopic women (age 18-40) Ocular findings? Bilateral white-yellow chorioretinal lesions (~100-200 microns) mostly posterior pole (FA: early hyper, late stain) No vitritis Risk of CNV Clinically similar to POHS, but symptomatic and negative Histoplasmin skin test Treatment & Prognosis? No treatment if no CNV Good prognosis if no CNV (50-75% of patients have BCVA>20/25)
78
Birdshot Chorioretinopathy
Usually occurs in middle-age (age 40-60) HLA Association? HLA-A29 (highest association 95%, but also 7% of healthy) Ocular findings? Biilateral cream-colored spots (50-100mm) in posterior pole, usually radiating from optic nerve FA findings? subtle compared with ophthalmoscopic appearance “quenching” (dye disappears quickly from vessels) Mild vitritis; vascular attenuation & leakage Optic disc edema or atrophy; CME Painless eye Decreased scotopic ERG Monitoring? CME & perivascular edema on OCT HVF & ERG Treatment & Prognosis? Early aggressive IMT (cyclosporine, azathioprine, MTX, Cellcept) Intravitreal triamcinolone Poor prognosis chronic & recurring

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