This document discusses inflammatory multfocal chorioretinopathies, which are a heterogeneous group of diseases that affect the outer retina, retinal pigment epithelium, choroid, or a combination. It summarizes the key characteristics, ocular manifestations, diagnosis, treatment, and outcomes of several specific conditions that fall under this category, including Birdshot chorioretinopathy, Acute posterior multifocal placoid pigment epitheliopathy, Serpiginous choroiditis, Multiple evanescent white dot syndrome, and Acute zonal occult outer retinopathy.

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

4.  Birdshot Chorioretinopathies (BCR)
 Acute Posterior Multifocal Placoid Pigment
Epitheliopathy (APMPPE)
 Serpiginous Choroiditis (SC)
 Multiple Evanescent White Dot Syndrome (MEWDS)
 Acute Zonal Occult Outer Retinopathy (AZOOR)

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

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

15. SYSTEMIC ASSOCIATIONS
 Cerebral vasculitis
 Cerebrospinal fluid pleocytosis

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

27. EPIEMIOLOGY AND PATHOGENESIS
Inflammatory chorioretinopathy affecting mainly young
healthy women in 2nd -4th decades of life

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

30. DIAGNOSIS
•Visual field testing
•Fluorescein angiography
•Indocyanine green
•OCT imaging
•Fundus autofluorescence
•ERG

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

37. Reference:
- Nussenblatt and whitecup 4th edition
- Myron yanoff 4th edition