Inflammatory chorioretinopathies Flashcards
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- Birdshot: Age 30-70 (older); F>M, Bilateral, 80-98% are HLA A29 positive. Insiduous onset, chronic, recurrent. Prognosis guarded without treatment. Therapy: systemic/local steroids; IMT.
Birdshot Symptoms
- Blurred vision, floaters, photopsias, disturbed color / night vision.
Birdshot Exam
- vitritis, ovoid creamy white-yellow post-equatorial lesions
Birdshot Complications
vasculitis, disc edema, CME, CNV (6%)
Birdshot FA
early hypofluorescence, late stain, leakage from disk, CNVM
Birdshot ICG
hypofluorescent lesions more numerous than on exam
Birdshot FAF
hypoautofluourescent lesions more numerous than clinically apparent; placoid macular hypoautofluoresence with central vision loss
Birdshot OCT
ME, loss of IS/OS, suprachoroidal fluid
Birdshot ERG
abnormal rod/cone response
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- APMPPE: Age 20-50 (young). M=F. Bilateral. Viral prodrome, cerebrovasculitis, CSF abnormalities. Course is acute and self-limited. Visual prognosis is good. Therapy is observation; steroids if CNS involvement.
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- Serpiginous Chorioretinitis: Age 20-60 (young-middle). M=F. Bilateral but asymmetric. Systemic association HLA B7. Rule out TB. Onset/course is variable and self-limited. Visual prognosis is guarded. Therapy is systemic/ local steroids and IMT. Anti-VEGF w/wo laser for CNVM.
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- Age 9-69 (usually more young). F>M (3:1). Bilateral. Onset/course is insidious, . chronic/recurrent. Visual prognosis: guarded. Treatment: steroids, IMT. Anti-VEGF, +/- laser for CNVM.
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PIC: age 18-40 (young). F (90%); bilateral. Onset/course is acute/self-limited. Prognosis is good if no CNVM. Treatment is systemic/local steroids, IMT, antivegf +/- laser
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SFU: subretinal fibrosis and uveitis syndrome. Age 14-34 (Young). F>M (>95%). Asymmetric. Insidious, chronic, recurrent. Prognosis is guarded. Treatment is steroids for CME if present
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- MEWDS: young (10-47); F (3:1), unilateral. Assc’n with viral prodrome in 50%. Course is acute, self limited. Prognosis is excellent. No treatment, just observation.
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- ARPE: young (16-40). M=F. Unilateral (75%). No associated systemic issues. Acute self-limited. Prognosis is excellent. No treatment.
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AZOOR: young. (13-63). F (3:1). Unilateral 24% of the time, bilateral 76% of the time. Associated with systemic autoimmune disease 28% of the time. Insiduous, chronic, recurrent (31% of the time). Prognosis is guarded. Treatment is steroids, IMT +/- antivirals
APMPPE symptoms
blurred vision, scotomata, photopsias
APMPPE exam
multifocal flat gray-white lesions 1-2 DD, RPE with evolving pigment
APMPPE complications
disc edema, pigmentary changes
APMPPE FA
acute lesions, early blockage, late staining, late window defects
APMPPE ICG:
hypofluorescent spots
APMPPE FAF
- hyperfluourscent areas corresponding to FA blockage and hypofluorescent areas corresponding to staining.
APMPPE OCT
outer retinal hyper-reflectivity with intra retinal and subretinal fluid
Serpiginous symptoms
blurred vision, scotomata
Serpiginous exam
- geographic yellow-gray macular chorioretinal lesions with centrifugal extension. Activity is at leading edge with atrophy in its wake.
Serpiginous complications
CNVM (25%), RPE mottling, scarring, loss of choriocapillaris
Serpiginous FA:
early hypofluorescence, late staining/ leak of active border/ CNVM
Serpiginous ICG
early hypofluorescence, late stain, more widespread than on exam
Serpiginous FAF
hyperfluorescent active lesions, hypofluorescent regressed lesions
Serpiginous OCT
- outer retinal hyperreflectivity in active lesions, RPE atrophy in regressed lesions
MCP symptoms
blurred vision, floaters, photopsias, metamorphopsias
MCP exam
- myopia, anterior uveítis (50%), vitritis (100%), active white-yellow lesions evolving to punched out scars.
MCP complications
- disc edema, peripapillary pigment changes, CME (14-44%), CNVM (33%).
MCP FA:
early block, late stain/leakage
MCP ICG
- many hypofluorescent lesions, confluence more around the nerve, more than on exam
MCP OCT:
sub-RPE deposits with overlying retinal distruption
MCP ERG
abnormal, extinguished responses
PIC symptoms:
paracentral; scotoma, photopsias, metamorphopsias
PIC Exam
myopia; vitritis is ABSENT; white-yellow chorioretinal lesions
PIC Complications
CNVM (17-40%), serous detachment over confluent lesions
PIC FA:
early block or HYPERfluorescence, variable late stain/leakage of acute lesions
PIC ICG:
hypofluorescent peripapillary/posterior pole lesions.
PIC FAF/OCT
similar to MCP. sub-RPE deposits with overlying retinal distruption
SFU symptoms
- blurred vision, decreased vision
SFU Exam:
moderate vitritis, yellow-white lesions in posterior pole to mid-periphery. RPE hypertrophy, atrophy, large stellate zones of subretinal fibrosis.
SFU complications
- retinal detachments, CME, CNVM
SFU FA
- alternating hyper/hypo fluorescent areas.
SFU OCT
variable edema, SR fluid, subretinal fibrosis
MEWDS symptoms
blurred/decreased vision, scotoma, photopsias
MEWDS exam
myopia, mild anterior uveitis, vitritis, small white-orange evanescent perifoveal dots.outer retina/RPE macular granularity.
MEWDS complications:
disc edema, venous sheathing
MEWDS FA
- early punctate hyperfluorescence, wreathlike configuration. Late staining of lesions/ optic nerve.
MEWDS ICG
- multiple hypofluorescent spots, more numerous than on exam.
MEWDS FAF:
- hyperfluorescent spots.
MEWDS OCT:
abnormal IS/OS
ARPE exam
small hyperpigmented lesions with yellow halos, unassociated vitritis
ARPE FAF
early hyperfluorescence with surrounding halo of hypofluorescence.
Azoor symptoms
photopsias, scotoma
Azoor exam:
- initially subtle RPE changes, late pigment migration, focal perifoveal sheathing.
Azoor complications
RPE mottling, occasional CME
Azoor OCT
loss of IS/OS.
- Inflammatory chorioretinopathies helpful hints (5):
- (1) all bilateral except SFU/serpiginous (asymmetric), MEWDS, (unilateral), ARPE (unilateral 75%), Azoor (unilateral 24% of the time, bilateral 76% of the time).
- (2) all can be seen younger except birdshot tends to be older
- (3) F>M except: APMPPE, serpiginous, ARPE (M=F). PIC is 90% F. SFU is >95% F.
- (4) PIC = NO vitritis. MCP = always vitritis
- (5) Associated CNVM: serpiginous (25%), MCP (33%), PIC (17-40%), SFU, Birdshot (6%)