White Dot Syndromes & Uveitis Flashcards
Quenching on FA
Birdshot
MEWDS
- 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.
Photopsias
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).
APMPPE
Block early stain late
Birdshot
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
AZOOR
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
Idiopathic blind spot enlargement syndromes
“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
Serpiginous
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.
PIC (punctate inner choroidopathy)
- 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
OHS vs. MCP
Both have punched out lesions
However, OHS has NO VITRITIS, as opposed to 90% MCP pts with vitritis.
MCP (multifocal choroiditis and panuveitis)
poor Px (like serpiginous. MC in females, affects OU
Type I
Anaphylactic or atopic
mast cells and histamine
Example? Allergic conjunctivitis seasonal component Atopic conjunctivitis environmental triggers less seasonal ezcema, asthma Anaphylaxis
Type II
Cytotoxic
Foreign or autoantigens
Example?
OCP (IgA deposits in BM)
Mooren Ulcer
Type III
Immune-complex reaction
Example? Scleritis Vasculitis Phacoanaphylaxis Stevens-Johnson syndrome
Type IV
Delayed hypersensitivity
T Cell mediated
Example? Contact dermatitis Phlyctenulosis Corneal graft rejection Sarcoid, TB
Type V
Stimulating antibody
Example?
Graves’ disease
Giant cells (3 types)
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
Anterior scleritis
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
Posterior scleritis (2%)
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
Reiter syndrome
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
Other seronegative dx w/ uveitis?
Ankylosing spondylitis
Psoriatic arthritis
Ulcerative colitis»_space; Crohn’s disease
Uveitis:
How long after quiescent for PK?
How long after quiescent to CE?
CE = 3 mo PK = 6 mo
Wegener Granulomatosis
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
Juvenile Idiopathic Arthritis
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
JIA Rx and Px
Treatment:
Steroids, immunomodulation
Avoid IOL after cataract surgery!
Worse prognosis if?
RF-, ANA+
Pauciarticular > Polyarticular
Lower limb joints > upper limb
Blau Syndrome
Familial juvenile systemic granulomatosis
Inheritance: AD
Multifocal choroiditis, vasculitic skin rash, polyarthritis, no pulmonary involvement
DDx of iritis + high IOP?
Fuchs Heterochromic Posner-Schlossman Sarcoidosis Herpetic uveitis Toxoplasmosis Syphilis
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
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)
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
Mikulicz syndrome?
- Dry eyes from lacrimal, salivary gland involvement
