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Posterior uveitis of unknown cause white spot syndromes
1. Bipin Bista
2nd Year Resident
Department of Ophthalmology
National medical College
& Teaching Hospital
2. A heterogeneous group of diseases that affect the outer
retina, retinal pigment epithelium(RPE),choroid or a
combination of these anatomic sites
The lesions are typically multifocal in nature
The may be present in one or both eyes
3. When bilateral they may be asymmetric
The white spots themselves may be a variable
finding.
For this reason, the name Inflammatory
Multifocal Chorioretinopathies may be more
appropriate
5. EPIDEMIOLOGY AND PATHOGENESIS
Primary lesions of the disease are in the choroid
Affects usually women between 3rd and 6th decades of
life
90% of the patient having BCR possess human
lymphocyte antigen A*29 (HLA-A*29)
6. OCULAR
MANIFSTATIONS
Blurred
vision,floaters,central and
peripheral photopsias and
later nyctalopia
Lesions are scattered around
the optic disk
Radiate to the equator in a
‘shot gun’ pattern
Creamy lesions are small
and less than 1 disk
diameter in size
Viterous inflammation,disc
edema,macular edema are
seen
7.
8. DIAGNOSIS
Fluorescein angiography reveals disc staining,vascular
leakage,and often late CME
Indocyanine green angiography
Optical coherence tomography(OCT)-it may cause vision
loss in BCR
Fundus autofluorescence(FAF) reveals more extensive
hypoautofluorescent lesions
Visual field abnormalities are common with BCR which
includes enlarged blind spot,central or paracentral
scotomas,generalized diminished sensitivity
9. TREATMENT
Corticosteroids are short term mainstay of therapy
They carry their own systemic and local
(glaucoma,cataract) risk
Fluocinolone acetonide
Systemic cyclosporine,azathioprine,low-dose
methotrexate
Anti-vascular endothelial growth factor(anti-VEGF)
are useful in the treatment of CNV associated with
inflammatory chorioretinal disorders
10. EPIDEMIOLOGY AND PATHOGENEIS
Bilateral inflammatory disease
Affecting choriocapillaries,RPE and outer retina in 2nd
and 3rd decades of life
11. OCULAR
MANIFESTATIONS
Sudden painless loss of
vision in one or more
typically both eyes
Transient hearing loss have
been reported
Cerebral vasculitis
No evidence of anterior
uveitis on Ocular
examination
Minimal to no vitreous cell
occur
Flat-to-placoid (plate like)
lesion of variable size
involving posterior pole
12. Lesions do not occur anterior to equator
Lesions of differing ages can be seen associated with
exudative detachments
Lesion tend to clear centrally and become
hypopigmented as they begin to resolve
Optic disc edema has also been noted in APMPPE
13. DIAGNOSIS
Clinical examination,time course and imaging
Placoid fundal lesion highly suggestive of APMPPE
Fluorescein angiography
Indocyanine green shows hypofluorescent lesions in
acute phase of disease
OCT imaging
Areas of hyper-reflectivity have been seen above RPE
in photoreceptor layer
16. PATHOLOGY
Unknown
Vascular insult leading Choroidal ischemia causing
RPE damage
Primary site of inflammation is in the outer retina
which then affects choroid
17. TREATMENT
No treatment is necessary, disease is self-limited
Systemic corticosteroids
Rarely CNV can develop
Anti-VEGF agents are useful in CNVs
18. COURSE AND OUTCOMES
Self-limited course of 2-6 weeks
Vision improves to near normal level during first 2-3
weeks
Complain of difficulty with reading or of scotomas in
the central visual field
Placoid lesion resolve over a period of 2-6 weeks
Permanent disruption of Bruch’s membrane and the
choriocapillaries occurs less frequently in APMPPE
than with serpigious choroidopathy
19. EPIDEMIOLOGY
Also called helicoid peripapillary chorioretinal
degeneration
Affects patients from 2nd to 7th decades
Men and women are affected equally
It is usually bilateral
Affects outer retina,RPE,choriocapillaries and large
choroidal vessels
20. OCULAR
MANIFESTATIONS
Bilateral disease
Symptomatic unilaterally
when the lesions affect the
fovea
Central scotoma with vision
loss
Lesions are geographic grey or
grey yellow begin in
peripapillary region or macula
Unlike APMPPE usually one
eye is active at a time with one
area of focus.
New lesions appear at the
edges of healed scars
Disease has progressive,step-
wise course
21. DIAGNOSIS
Fluorescein angiography demonstrates early
hypofluorescense and late hyperfluorescense of active
lesions
Indocyanine green angiography shows hypofluorescent
active lesions
Choroidal neovascularization shows late leakage and
often arises from borders of old scars
22. Association of Serpiginous choroiditis and tuberculosis
has been suggested
However, treatment of non-TB patients with
antimicrobial agents has made no difference in course
of these patients
23. PATHOLOGY
Loss of RPE with destruction of overlying retina and
lymphocyte
Pathogenesis is unknown
24. TREATMENT
Relapsing and progressive in nature
Therapy is aimed at treating acute episodes and
preventing recurrence that can lead to foveal involvement
Steroids(routes:intravenous,intravitreal,subtenon’s and
via implant)
Aggressive management with corticosteroids used to
treat acute attacks not preventing recurrence
Cyclosporine,azathioprine and other cytotoxic agents
25. Assistance of rheumatologist,oncologist is required due
to its potential systemic side-effects
Anti-VGEF injection
Photodynamic therapy and thermal laser for extra
foveal CNV
Oral acetazolamide for CME
26. COURSE AND OUTCOMES
Foveal destruction may occur
Central visual acuity is lost in 20% or more
Central visual loss
28. OCULAR MANIFESTATIONS
Acute unilateral painless visual loss
Scotoma associated shimmering photopsias, often in
temporal visual field
Numerous,small white spots concentrated in
paramacular area, less prominent beyond vascular
arcades
29. Granular appearance to
fovea is usually present,
fovea does not return to
normal appearance
Vitritis and disc edema
Retinal vascular
sheathing
Circumpapillary
discoloration or white
lesion is the presenting
sign
31. COURSE AND OUTCOMES
Self-limited course
White dots fade and edema resolves within 2-6 weeks
of onset of symptoms
Visual acuity returns to baseline levels
Recurrences are uncommon but have been reported
Visual prognosis is good
Uncommon association of MEWDS with acute macular
neuroretinopathy has been described but unclear
Rare association with AZOOR
32. EPIDEMIOLOGY
Affects young healthy women in 2nd-4th decades of life
Photopsias and dense scotomas
Unilateral or bilateral
Systemic autoimmune disease have been noted in some
patients
33. OCULAR MANIFESTATIONS AND DIAGNOSIS
Fundus finding are usually normal.
Narrow retinal vessels and depigmentation of the
RPE.
Sharp demarcation between normal and abnormal
appearance showing curvilinear appearance.
34. Typically normal.
RPE occur with pigment mottling & window mottling.
A grey white line is seen between normal and
abnormal retina.
OCT irregularity of the IS-OS photoreceptor line in the
areas of retinal involvement.
RPE & Outer retina usually atrophies.
Visual field – superior, temporal and sometimes central
visual field loss.
ERG – severe reduction in a-wave amplitude
35. May show progression and regression with new areas
of involvement.
No specific treatment.
36. Three types of AZOOR exist
1. Type 1 : no other associated WSS.
2. Type 2 : Begins with another WSS
3. Type 3 : No clinical retinal abnormalities are seen but
photoreceptor dysfunction is evidenced on OCT/ERG