A Review of the Inflammatory Chorioretinopathies: The White Dot Syndromes

Table 1

	ARPE	MEWDS	APMPPE	PIC	MCP	AZOOR	Birdshot	SFU	Serpiginous Choroidopathy
Sex	M = F	F > M	M = F	F > M	F > M	F > M	F > M	F > M	M > F
Age	Young (10 s–30 s)	(20 s–50 s)	(20 s–30 s)	Young (≤40)	(20 s–60 s)	Young women	(40 s–60 s)	(10 s–30 s)	(30 s–60 s)
Laterality	Variable (Uni-75%)	Mostly unilateral	Bilateral	Bilateral	Bilateral	Bilateral but can be asymmetric	Bilateral	Bilateral (asymmteric)	Bilateral but usually asymmetric
Onset	Sudden	Acute	Acute	Sudden	insidious	Sudden	Insidious	Acute	Variable
Viral Prodrome	Variable	Variable	Variable	None	Variable	Variable	None	None	None
Symptoms	Decreased visual acuity or central metamorphopsia or scotoma	Blurred vision, scotomas, photopsias	Blurred vision, scotomata, Photopsia	Decreased central visual acuity, Photopsias, Scotomata	Blurred vision, scotomata, photopsias, and floaters	Visual field defect, blurred vision, photopsias, whitening of vision	Blurred vision, floaters, difficulty with night vision/or color vision, photopsias	Decreased vision	Blurred vision, paracentral or central scotomata, photopsias
Duration	Weeks-Months	Weeks-months	Weeks-Months		Chronic	Weeks to months	Chronic	Chronic	Chronic
Recurrence	Variable	Rare	Rare	Rare	Recurrent	Variable	Recurrent	Recurrent	Recurrent acute lesions lasting weeks-months
Findings	Small hyperpigmented lesions (100–200 𝜇m)	Myopia, small white dots in outer retina/RPE, may coalesce to form patches, disc edema, white/orange granularity at level of fovea and enlargement of blind spot	Multifocal, Flat, Gray-white placoid lesions at the level of posterior pole RPE improving within 1-2 weeks, may have disc swelling	Small (100–300 mm in diameter) multiple gray or yellow, opaque round lesions at the level of the RPE-choroid, scattered throughout the posterior pole, evolve usually into atrophic chorioretinal scars, may be complicated by CNV or subretinal fibrosis	Myopia, Iritis in 50%, Yellow-white lesions replaced by punched out scars, +/− disc swelling	Normal appearing fundus or some RPE mottling or zones with retinitis pigmentosa (RP) appearance	multiple, ill-defined cream-colored lesions at level of outer retina/RPE, patches of depigmentation, otic atrophy and some disc swelling	Blurred and decreased vision	Pseudopodial/geographic zone of gray-yellow discoloration of RPE in peripapillary/macula area with centrifugal extension with active and peripheral edge at the RPE and choriocpillaris
Vit Cells	Mild/none	Mild	Mild	None	Moderate	Normal to mild vitritis	Moderate	AC and Vitreous inflammation	Mild versus absent
FA	Early hyperfluorescence, late staining	Early hyperfluorescence with punctate leisons in wreathlike configuration, late staining with	Early block with late staining in acute phase of disease and window defects in later stage of disease process	Early block with late staining in early phase of disease and window defects in later stage of disease process	Early block with late staining in acute phase of disease and window defects in later stage of disease process	Normal angiography to variable changes to include hyperfluorescence, window defects and optic nerve head leakage.	May have vascular leakage	Yellow-white lesions (50–500 𝜇m) in the posterior pole to midperiphery, RPE hypertrophy, and atrophy and stellate zones of subretinal fibrosis	Hypofluorescent early, borders stain late
ERG/EOG	Normal ERG with Abnormal EOG	Abnormal ERG	Abnormal EOG	Normal-mild changes in ERG	Normal-abnormal ERG	Abnormal ERG	Abnormal rod and cone ERG	Abnormal ERG/EOG	Normal
CME/CNV	None	Rare	None	1/3 develop CNV	CME may be seen with CNV	CME Rare	CME, rare CNV	CME	CNV rare but can occur at margin of chorioretinal atrophy.
Treatment	None	Observation	Observation; consider corticosteroids with CNS involvement	Observation versus oral/periocular corticosteroids	Corticosteroids; photocoagulation. PDT/Anti-VEGF for CNV	No treatment known to improve symptoms	Cyclosporine, Mycophenolate, methotrexate, IVIG	Corticosteroids for CME, immunomodulat-ory therapy	Immunosupression, antivirals, Photocoagulation for CNV
Prognosis	Excellent	Very Good	Good	Good	Generally poor	Good, stabilization of visual fields defects usually within 6 months of onset	Guarded	Guarded	Guarded
Etiology	Unknown	viral	viral	Autoimmune	Viral	Autoimmune	Autoimmune	Autoimmune	viral, autoimmune,
Sequelae		Mild RPE changes	RPE mottling/; depigmentation	Scarring, CNV (30%)	Punched out scars,		CME, rare CNV	Venous sheathing, disc edema	RPE mottling, scarring, loss of choriocapillaris, CNV
HLA	None	None	HLA-B7, HLA-DR2	None	None	None	HLA-A29 (strong)		HLA-B7, S-antigen