White Dot Syndromes

Key Points at a Glance

Key Points at a Glance

• White dot syndromes (WDS) are a group of inflammatory diseases primarily affecting the outer retina, retinal pigment epithelium (RPE), choriocapillaris, and choroidal stroma.
• Seven representative diseases: APMPPE, MEWDS, PIC, MFC (MFCwP), birdshot chorioretinopathy, serpiginous choroiditis, and AZOOR.
• They are broadly classified into “young women, unilateral, spontaneous resolution” type (MEWDS, PIC, AZOOR) and “middle-aged to elderly, bilateral, chronic progressive” type (birdshot, serpiginous).
• Multimodal imaging (OCT, FA, ICGA, FAF, OCT-A) is central to differential diagnosis and activity assessment, with each disease showing characteristic patterns.
• The risk of CNV (choroidal neovascularization) is particularly high in PIC (40–76%), MFC (up to 60%), and serpiginous choroiditis (up to 35%).
• Treatment strategies vary greatly by disease: spontaneous remission (MEWDS, APMPPE) to long-term immunosuppression (Birdshot, Serpiginous).
• Differentiation from tuberculosis (especially serpiginous choroiditis) is essential for treatment and must be ruled out.

1. What are white dot syndromes?

White dot syndromes, a concept named by Gass in 1977, refer to a group of inflammatory diseases characterized by multiple white or yellowish-white spotty lesions in the fundus. Although the definition of target diseases varies among researchers, it is now widely used as a group of non-infectious idiopathic inflammatory diseases primarily involving the outer retina, RPE, choriocapillaris, and choroid ¹⁾.

In the Uveitis Clinical Practice Guidelines (Jpn J Ophthalmol 2019;123(6):635-696), APMPPE, MEWDS, PIC, multifocal choroiditis, birdshot retinochoroidopathy, serpiginous choroiditis, and AZOOR are listed as independent diseases under the classification of posterior uveitis, and when the inclusive term “white dot syndromes” is used, it encompasses these diseases ²⁾.

Classification concepts

With recent advances in multimodal imaging (including OCT-A), white dot syndromes are now classified into the following three groups based on the primary layer of involvement¹⁾.

Outer retina predominant type

MEWDS・AZOOR

• Primary involvement is transient disruption of the ellipsoid zone (photoreceptor inner/outer segment junction)
• Choriocapillaris is generally spared (no to minimal flow void on OCT-A)
• Highly reversible with a strong tendency for spontaneous resolution

Choriocapillaris-dominant type

APMPPE, Serpiginous choroiditis, PIC

• Occlusive vasculitis of the choriocapillaris is central to the pathology
• OCT-A shows choriocapillaris flow void
• Outer retinal and RPE changes are secondary

Choroidal stroma-dominant type

Birdshot chorioretinopathy

• Primary lesion is lymphocytic infiltration of the choroidal stroma
• OCT-A shows flow void in Haller’s layer, with initial sparing of choriocapillaris
• Chronic progressive course with strong association with HLA-A29

Furthermore, the concept of AZOOR complex has been proposed, integrating MEWDS, AZOOR, PIC, MFC, AMN, AIBSE, and AAOR as a continuum with a common genetic autoimmune/inflammatory basis³⁾.

Epidemiology in Japan

According to statistics from the Japanese Society for Ocular Inflammation, the proportion of each white dot syndrome among all uveitis cases is as follows²⁾.

Disease	Proportion of all uveitis
MEWDS	Approximately 1–2% (domestic reports)
APMPPE	Rare (no clear annual incidence statistics)
PIC	Rare
Serpiginous choroiditis	Approximately 0.3%
Birdshot retinochoroidopathy	Rare (more common in Caucasians, extremely rare in Japan)
AZOOR	Rare (increasing reports in recent years)

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2. Common clinical features

Although symptoms vary by disease, the following clinical features are common to the white dot syndromes^{1, 2)}.

Common patterns of subjective symptoms

• Decreased visual acuity: ranges from mild (MEWDS, AZOOR) to severe (serpiginous choroiditis, PIC with CNV)
• Photopsia: the most common symptom reflecting outer retinal/photoreceptor damage
• Scotoma/visual field defect: often paracentral or central scotomas corresponding to lesion location
• Metamorphopsia: occurs with macular lesions or CNV

Presence of anterior segment inflammation

Disease group	Anterior chamber/vitreous inflammation
MEWDS, APMPPE, PIC	Usually none (APMPPE may have mild)
MFC (MFCwP)	Anterior chamber inflammation + vitritis present (differentiating from PIC)
Birdshot	No to minimal anterior chamber inflammation, vitritis present
Serpiginous choroiditis	Anterior chamber inflammation and vitritis are usually mild
AZOOR	Usually none

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3. Differential diagnosis approach

Age, Sex, Unilateral vs Bilateral, and Recurrence-Based Classification

Young women, unilateral, spontaneous resolution

MEWDS · AZOOR · (some PIC)

• Common in myopic women in their 20s–40s
• Often unilateral (MEWDS is >95% unilateral)
• Tends to recover spontaneously with observation
• Annual incidence of MEWDS: approximately 0.22 cases per 100,000 people

Young to middle-aged women, bilateral, CNV risk

PIC·MFC

• Myopic women in their 20s to 40s (female ratio >90%)
• Often bilateral (80% in PIC)
• Leave atrophic scars and have a high rate of CNV complication
• PIC complicated with CNV: 40–76%

Young to middle-aged, bilateral, acute onset

APMPPE

• Peak incidence in 20s–30s (mean age 25), no sex difference
• Bilateral, acute, tendency for spontaneous resolution
• Watch for cerebral vasculitis (urgent evaluation if neurological symptoms appear)

Middle-aged and older adults, bilateral, chronic progressive

Birdshot・Serpiginous choroiditis

• Birdshot: 40s–60s, slightly more common in women
• Serpiginous: 30s–50s, slightly more common in men
• Both diseases are chronic, recurrent, and require long-term immunosuppression
• HLA-A29 positivity rate in Birdshot (Caucasians): 80–98%

Differential Diagnosis Flowchart

眼底に白点状病変
      │
      ├─ 片眼性？
      │    ├─ YES → MEWDS・AZOOR・AMN を考慮
      │    │         ↳ FA で初期過蛍光 → MEWDS
      │    │         ↳ 眼底ほぼ正常・ERG 異常 → AZOOR
      │    └─ NO（両眼性）
      │
      ├─ 急性発症・後極部大型白斑？
      │    └─ YES → APMPPE（FA 蛍光逆転現象を確認）
      │
      ├─ 後極部小病変・近視女性・硝子体炎なし？
      │    └─ YES → PIC を考慮（CNV 検索：OCTA 必須）
      │
      ├─ 小病変・硝子体炎あり・周辺部にも病変？
      │    └─ YES → MFC（MFCwP）を考慮
      │
      ├─ 乳頭周囲から蛇行状に進展・男性多め？
      │    └─ YES → 蛇行状脈絡膜炎（結核除外が最優先）
      │
      └─ 後極部散弾状病変・中高年・HLA-A29？
           └─ YES → Birdshot 網脈絡膜症

Important: Exclude Tuberculosis

If serpiginous choroiditis is suspected or similar multifocal choroidal lesions are observed, always perform tuberculosis exclusion testing using IGRA (QuantiFERON) or TST (tuberculin skin test). Because using immunosuppressive drugs for tuberculous serpiginous-like choroiditis (SLC) can markedly worsen tuberculosis, the treatment strategy is fundamentally different²⁾.

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4. Comparison of Major Diseases

Main Comparison Table: 7-Disease Matrix

Item	APMPPE	MEWDS	PIC	MFC (MFCwP)	Birdshot	Serpiginous choroiditis	AZOOR
Typical age of onset	20s–30s (average 25 years)	20s–50s	18–40s (average 36 years)	Average 30 years	40s–60s	30s–50s	Mainly mid-30s
Sex	No sex difference	Female predominance (1:4)	Female predominance (approx. 90%)	Female predominance (more common in white women)	Slightly more females	Slightly more males	Female predominance (approx. 75%)
Unilateral/Bilateral	Mostly bilateral	Unilateral (≥95%)	Mostly bilateral (80%)	Bilateral	Bilateral	Bilateral	Unilateral → bilateral progression (eventually 76% bilateral)
Main symptoms	Decreased vision, central scotoma, metamorphopsia	Decreased vision, photopsia, blurred vision	Scotoma, decreased vision, metamorphopsia	Floaters, decreased vision, photopsia	Decreased vision, night blindness, color vision abnormality	Decreased vision, paracentral scotoma	Photopsia, visual field defect (fundus nearly normal)
Characteristics of fundus white spots	Large cream-colored white spots in the posterior pole (1/4 to 1/2 disc diameter)	Pale gray-white multiple small spots from posterior pole to equator (100–200 μm)	Yellow-white small spots in the posterior pole (100–300 μm), 12–25 in number	Yellow-gray spots from posterior pole to periphery (45–350 μm), with vitreitis	Shotgun-like cream-colored spots from posterior pole to equator (1/4 to 1/2 disc diameter)	Geographic gray-yellow lesions extending tortuously from the peripapillary area	Fundus nearly normal (acute phase), outer layer atrophy in late phase
OCT findings	Ellipsoid zone disruption + outer retinal hyperreflectivity, partial atrophy after recovery	Ellipsoid zone marked disruption/disappearance (acute phase) → recovery	Sub-RPE hyperreflective elevation + EZ break (5-stage evolution)	Sub-RPE hyperreflectivity + EZ break (similar to PIC)	Choroidal lesions, cystoid macular edema, EZ disappearance indicates poor prognosis	Active phase: outer retinal hyperreflectivity, subretinal fluid. Scar phase: RPE atrophy	EZ (IS/OS) disappearance is the most important finding (corresponds to visual field defect)
FA findings	Early hypofluorescence → late hyperfluorescence (fluorescence reversal)	Persistent wreath-like hyperfluorescence from early phase	Active: early arterial hyperfluorescence → late leakage	Active: early hypofluorescence → late leakage	Lesion hyperfluorescence (no leakage) + vascular leakage and optic disc hyperfluorescence	Active: early hypofluorescence → late hyperfluorescence (leakage)	Usually normal or minimal abnormalities
ICGA Findings	Hypofluorescence in all phases (directly reflects choriocapillaris ischemia)	Late hypofluorescence (more extensive than white dots)	Mid-phase hypofluorescence, useful for detecting subclinical lesions	Early to all-phase hypofluorescence	Early/mid hypofluorescence → late isofluorescence (initial) → advanced: all-phase hypofluorescence	All-phase hypofluorescence (reflects choroidal circulatory disturbance)	Usually normal
FAF findings	Acute: hypo- or hyper-autofluorescence. Remission: hypo-autofluorescence	Acute: hyper-autofluorescence (multiple hyperfluorescent spots). After recovery: normalization	Active: hypo-autofluorescence (hypoAF), marginal hyper-autofluorescence halo	Active: hypo-autofluorescence	Peripapillary confluent hypo-autofluorescence (seen in 73%)	Active: hyper-autofluorescence margin + hypo-autofluorescence halo. Inactive: hypo-autofluorescence	Zonal hyper- to hypo-autofluorescence abnormalities
OCT-A findings	Choriocapillaris flow void (high concordance with FA/ICGA)	Choriocapillaris generally preserved (some transient flow void)	Choriocapillaris flow void (at sites of inflammatory lesions)	Choriocapillaris flow void	Haller layer flow void (early) → advanced: full-thickness flow void	Choriocapillaris flow void (severe)	Choriocapillaris generally preserved
Recurrence	Rare (basically single episode)	Recurrence in about 10%	High (chronic recurrent)	High (recurrent inflammatory episodes)	High (chronic with repeated relapses and remissions)	High (repeats at intervals of 3 months to 4 years)	Most stabilize within 6 months; some progress
CNV complication rate	Rare	Rare	40–76% (high risk)	Up to 60%	Subretinal CNV: Rare	Up to 35%	Almost none
HLA associated	None	HLA-B51 (preliminary reports)	HLA-DR2, HLA-DRB1*15	IL-10 haplotype association	HLA-A29 (80–98% in Caucasians)	HLA-B7, HLA-A2 (reported associations)	None (immunological predisposition)
Treatment strategy	Observation (spontaneous resolution), severe: steroids	Observation, severe: short-term steroids, CNV: anti-VEGF	Observation (no CNV), CNV: anti-VEGF + steroids, immunosuppressants	Steroids + immunomodulatory therapy, CNV: anti-VEGF	Steroids + mycophenolate mofetil / adalimumab (long-term)	Steroids + immunosuppressants (including alkylating agents), CNV: anti-VEGF	Observation, severe: steroid pulse
Visual prognosis	Good (often spontaneous resolution)	Good (caution for recurrence and CNV)	High risk of poor prognosis if CNV develops	Risk of poor prognosis due to CNV and macular edema	Without treatment, 16–22% have visual acuity ≤0.1 at 10 years	Irreversible if fovea involved; up to 25% of eyes have final visual acuity <20/200	Mostly stable. Poor prognosis in cases with progressive outer layer damage

Q Which disease among the white dot syndromes has the worst visual prognosis?

A

Serpiginous choroiditis and birdshot retinochoroidopathy have the poorest visual prognosis. In serpiginous choroiditis, up to 25% of eyes have a final visual acuity of less than 20/200, and in birdshot, without treatment, 16–22% of patients have visual acuity of 0.1 or less over 10 years^{2, 4)}. PIC and MFC have a higher risk of poor outcomes when complicated by CNV. APMPPE and MEWDS have a strong tendency for spontaneous resolution and a good prognosis.

Detailed Clinical Features of Each Disease

APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy)

APMPPE commonly occurs in individuals in their 20s to 30s (average age 25) with no gender predilection. It is thought to be caused by occlusive vasculitis of the afferent arterioles of the choriocapillaris, and viral infections are suspected as triggers^{1, 2)}.

Prodromal symptoms and course

• About half of patients have cold-like symptoms (influenza, EB virus, chickenpox, streptococcal infection, etc.)
• Multiple cream-colored disc-shaped white spots, 1/4 to 1/2 optic disc diameter, appear in the posterior pole of both eyes
• The white spots begin to fade from the center within a few days and disappear after 7–12 days, leaving mild depigmentation
• Usually a single episode resolves spontaneously (recurrence is rare)
• Visual prognosis is generally good, but may be poor in severe cases or those progressing to geographic choroiditis

Specific complication: Cerebral vasculitis (MCAT)

APMPPE complicated by central nervous system vasculitis (MCAT: multiple cerebral arterial thrombosis) is a serious complication. If headache, fever, or neurological symptoms appear, emergency brain MRI and MRA should be performed. In cases with cerebral vasculitis, methylprednisolone pulse therapy and collaboration with neurology are necessary²⁾.

Relationship with the placoid chorioretinitis spectrum

APMPPE, together with PPM (persistent placoid maculopathy) and RPC (relentless placoid chorioretinitis), forms the “placoid chorioretinitis spectrum,” with choriocapillaris ischemia as a common pathological basis⁵⁾.

Oliveira MA, et al. Management of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Insights from Multimodal Imaging with OCTA. Case Rep Ophthalmol Med. 2020. Figure 5. PMCID: PMC7094199. License: CC BY.

Multiple geographic and placoid lesions in the posterior pole of both eyes appear as hypofluorescence on ICGA, areas of decreased choriocapillaris flow on OCTA, and hyperreflective changes from the outer plexiform layer to the RPE on OCT. These correspond to the multimodal findings of placoid lesions discussed in the section “APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy).”

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MEWDS (Multiple Evanescent White Dot Syndrome)

MEWDS predominantly affects women in their 20s to 50s (male-to-female ratio 1:4) and is characterized by unilateral, acute onset, and spontaneous resolution.

Characteristic clinical features

• Pale gray-white multiple small spots (100–200 μm) at the deep retinal to RPE level in the posterior pole to the equator
• Foveal granularity: observed in 74–96% of patients, may be the only residual finding after white spots disappear. Near-infrared FAF (NIR-FAF) shows a characteristic pattern⁹⁾
• Orange-dot appearance: characteristic finding on fundus photography and near-infrared fundus imaging
• Ivory lesion: A faint, blurred white change at the posterior pole of the fundus
• Approximately 50% of cases are preceded by cold-like prodromal symptoms
• Annual incidence is about 0.22 per 100,000 people, with recurrence in 10% of cases

Wreath-like hyperfluorescence on FA

Characteristic wreath-like hyperfluorescence from the early phase of FA is a key point in diagnosing MEWDS. The white spot lesions show hyperfluorescence from the early phase of FA and do not enlarge in the late phase. This early hyperfluorescence is an important distinguishing feature from the early hypofluorescence (fluorescence reversal phenomenon) of APMPPE^{1, 9)}.

Continuity with AZOOR complex

MEWDS primarily involves transient destruction of the ellipsoid zone (IS/OS line) of photoreceptors and is understood as a disease belonging to the AZOOR complex. OCT can confirm disruption or loss of the ellipsoid zone in the acute phase, which improves with visual recovery³⁾.

Papasavvas I, et al. Choroidal vasculitis as a biomarker of inflammation of the choroid. Indocyanine Green Angiography (ICGA) spearheading for diagnosis and follow-up, an imaging tutorial. J Ophthalmic Inflamm Infect. 2024. Figure 5. PMCID: PMC11618284. License: CC BY.

Pale white dot lesions are scattered at the posterior pole, with punctate blood flow abnormalities on FA and ICGA, and corresponding hyperautofluorescence on FAF. This corresponds to the multimodal findings of FA wreath-like hyperfluorescence and ICGA hypofluorescent spots discussed in the section “MEWDS (Multiple Evanescent White Dot Syndrome).”

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PIC (Punctate Inner Choroidopathy)

PIC predominantly occurs in young women (approximately 90%) aged 18–40 years with myopia (average around -5 D).

Characteristic clinical features

• Yellow-white small spots of 100–300 μm confined to the posterior pole, usually 12–25 in number
• No anterior chamber inflammation or vitritis (this is the most important distinguishing feature from MFC)
• Active lesions can be seen on OCT as hyperreflective elevations beneath the RPE
• Scarring leaves small atrophic lesions

CNV complication (40–76%) is the major clinical problem

The most important complication of PIC is CNV, with reported rates of 40–76%^{7, 8)}. CNV is more likely to occur due to the following factors:

• Bruch membrane fragility due to myopic choroidal thinning
• Bruch membrane disruption due to sub-RPE inflammation
• Increased local production of inflammatory cytokines (e.g., VEGF)

OCT-A has been shown to be more sensitive than FA for CNV screening, and regular OCT-A monitoring is recommended. Sudden worsening of metamorphopsia is a sign of CNV development and requires prompt evaluation.

Association with systemic diseases

Coexistence of PIC and sarcoidosis has been reported; in cases with multiple lung lesions, perform chest CT, serum ACE, and lysozyme tests. Association with HLA-DR2 and HLA-DRB1*15 has also been reported³⁾.

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MFC (Multifocal Choroiditis, MFCwP)

MFC (multifocal choroiditis with panuveitis; MFCwP) is on the same spectrum as PIC, but the key distinguishing feature is the presence of vitritis and anterior chamber inflammation⁷⁾.

Characteristic clinical features

• Multiple yellow-gray spots measuring 45–350 μm appear not only in the posterior pole but also in the mid-periphery.
• Characterized by a chronic recurrent course (repeated inflammatory episodes)
• Epiretinal membrane (ERM) is frequently associated (up to 35%), affecting long-term visual prognosis
• In some cases, inflammation cannot be controlled without immunosuppressive therapy

Treatment considerations

MFC tends not to resolve spontaneously, and many cases require long-term immunomodulatory therapy. When steroids alone are insufficient, methotrexate (MTX), azathioprine (AZA), or mycophenolate mofetil (MMF) are used. When CNV is present, a dual approach with anti-VEGF therapy and immunomodulatory therapy is important^{7, 8)}.

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Birdshot Chorioretinopathy (Birdshot Retinochoroidopathy)

Birdshot typically occurs in middle-aged to older adults aged 40–60 (average 50s), with a slight female predominance (1.5:1). It is more common in Caucasians and has one of the strongest genetic associations among known diseases, linked to HLA-A29 (relative risk 50–224 times in Caucasians)⁴⁾.

Characteristic fundus findings

• Multiple cream-colored spots (1/4 to 1/2 disc diameter) resembling birdshot, symmetrically distributed from the posterior pole to the equator
• Spots transition to scar lesions without pigmentation
• May be accompanied by retinal vasculitis and optic disc swelling

Characteristic functional changes

• Night blindness and color vision abnormalities: Appear early and may precede visual acuity loss
• Negative-type full-field ERG: Observed early, with a-wave amplitude decreasing as the disease progresses
• Delayed 30-Hz flicker ERG: The most sensitive indicator for monitoring activity; can detect abnormalities earlier than visual acuity loss¹⁷⁾

Precautions in Japanese patients

Because the frequency of HLA-A29 is low in Japanese individuals, the diagnostic sensitivity of HLA-A29 is limited. Diagnosis should emphasize clinical findings from the SUN 2021 classification criteria (birdshot fundus lesions, minimal anterior chamber inflammation, and presence of vitritis)¹⁰⁾.

Long-term complications

• Cystoid macular edema (CME): main cause of vision loss
• Optic disc swelling and optic atrophy
• With steroid implants (fluocinolone), intraocular pressure elevation occurs in up to 40% of cases, sometimes requiring trabeculectomy

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Serpiginous choroidopathy

Serpiginous choroidopathy is a bilateral chronic choroiditis that occurs in the 30s to 50s (slightly more common in men), characterized by geographic gray-yellow lesions that extend in a serpentine pattern from the peripapillary area.

Characteristic progression pattern

• Starts centripetally from the peripapillary area, with the lesion margin gradually expanding in a serpentine pattern
• Active phase: a gray-white border appears at the lesion margin
• Scar phase: becomes fixed as chorioretinal atrophic lesions
• During recurrence, new inflammation always appears from the margin of the existing scar (this is characteristic)
• The recurrence interval varies greatly among individuals, ranging from 3 months to 4 years.

Most important: Differentiation from tuberculosis-associated type (SLC)

Tuberculous serpiginous-like choroiditis (SLC) closely resembles serpiginous choroidopathy in imaging findings, but the treatment strategy is fundamentally different:

Differentiating feature	Serpiginous choroidopathy	Tuberculosis-associated type (SLC)
Lesion distribution	Peripapillary, centripetal	Posterior pole to periphery, multifocal
IGRA/TST	Negative	Positive
Lesion shape	Geographic, continuous	Multiple discontinuous small lesions common
Treatment	Steroids + immunosuppressants	Antitubercular drugs are essential

Since immunosuppressive drugs can markedly worsen tuberculosis in SLC, IGRA (QuantiFERON) testing before treatment is absolutely essential²⁾.

Management of CNV (up to 35%)

In serpiginous choroidopathy, CNV occurs in up to 35% of cases and can cause irreversible vision loss if it involves the fovea. Intravitreal injection of anti-VEGF therapy (bevacizumab, ranibizumab) is effective¹⁸⁾.

Macedo S, et al. Optical coherence tomography angiography (OCTA) findings in Serpiginous Choroiditis. BMC Ophthalmol. 2020. Figure 1. PMCID: PMC7325353. License: CC BY.

Fundus autofluorescence, fluorescein angiography, and OCT show serpiginous chorioretinal lesions spreading centrifugally from the optic disc. This corresponds to the peripapillary serpiginous progression pattern discussed in the section “Serpiginous choroidopathy.”

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AZOOR (Acute Zonal Occult Outer Retinopathy)

AZOOR is a disease concept proposed by Gass in 1992. It is an outer retinopathy that causes sudden vision loss, visual field defects, and photopsia despite a nearly normal fundus³⁾.

Concept of AZOOR complex

The AZOOR complex, proposed by Jampol et al., is a concept that understands MEWDS, AZOOR, PIC, MFC, AMN (acute macular neuroretinopathy), AIBSE, and AAOR as a continuum with a common genetic autoimmune/inflammatory basis³⁾.

Characteristic clinical features

• Frequently occurs in young women in their 20s to 50s with myopia.
• Photopsia (light perception) often appears in the early stage (especially light seen as带状 or arc-shaped)
• Starts unilaterally, eventually becomes bilateral in 76% of cases
• Fundus is almost normal in the acute phase (characterized by discrepancy between visual acuity loss and fundus findings)
• Visual field defects show irregular带状 patterns (often continuous with blind spots)
• May be associated with autoimmune diseases (Hashimoto’s disease, multiple sclerosis)

OCT and ERG are key to diagnosis

• The most important finding on OCT is disappearance or obscuration of the ellipsoid zone (IS/OS line).
• Functional recovery cannot be expected in areas where the outer layer has disappeared on OCT (also useful for prognosis prediction).
• Multifocal ERG can detect amplitude reduction even when the fundus appears normal (multifocal ERG is more sensitive than full-field ERG).
• Infrared FAF may delineate the border between the lesion and normal area.

Treatment and Prognosis

There is no established treatment for AZOOR. Mild cases may only require observation, but severe cases (with decreased visual acuity or extensive visual field defects) are treated with methylprednisolone pulse therapy (1,000 mg × 3 days) followed by oral prednisolone²⁾. Most cases stabilize within 6 months, but visual field defects in areas with residual outer layer damage do not recover.

Q How do you differentiate AZOOR from retrobulbar optic neuritis?

A

Both AZOOR and retrobulbar optic neuritis present with nearly normal fundus and decreased visual acuity and visual field, so differentiation is necessary. Key points for differentiation are: ① AZOOR shows reduced multifocal ERG amplitudes, while retrobulbar optic neuritis has normal ERG; ② AZOOR exhibits irregular带状 or arcuate visual field defects, whereas retrobulbar optic neuritis often presents with central scotomas; ③ AZOOR usually has mild RAPD, while retrobulbar optic neuritis shows marked RAPD; ④ On OCT, AZOOR shows ellipsoid zone loss, while retrobulbar optic neuritis shows optic disc swelling or RNFL thinning, which aids differentiation^{2, 3)}.

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5. Use of Imaging Tests

Role Allocation by Modality

Clearly understanding the role of each imaging test is essential for accurate diagnosis and activity assessment of white dot syndromes^{1, 5)}.

Modality	Strongest indication/role
FA (fluorescein angiography)	Evaluation of leakage from retinal vessels, RPE, and choriocapillaris. Confirmation of fluorescence reversal in APMPPE. Confirmation of wreath-like hyperfluorescence in MEWDS. Evaluation of vasculitis (Birdshot).
ICGA (indocyanine green angiography)	Direct evaluation of choroidal circulation disorders. Detection of lesions earlier than FA (especially Birdshot, APMPPE, serpiginous). Detection of lesions more extensive than clinical findings (MEWDS, PIC). Most sensitive for detecting active lesions.
FAF (Fundus Autofluorescence)	Noninvasive assessment of RPE damage. Activity evaluation (APMPPE, serpiginous). Diagnosis of MEWDS (early white dots show hyperautofluorescence). Chronicity assessment of Birdshot (peripapillary hypoautofluorescence 73%)
OCT (Optical Coherence Tomography)	Ellipsoid zone evaluation (diagnostic finding in MEWDS, AZOOR). Five-stage evolution assessment of lesions (PIC). Evaluation of CNV and macular edema. Prognosis prediction (EZ loss → poor visual prognosis)
OCT-A	Noninvasive detection of choriocapillaris flow void (APMPPE, serpiginous, PIC). Early and sensitive detection of CNV (higher sensitivity than FA in PIC, MFC). Layer-specific choroidal blood flow assessment in Birdshot. Treatment effect monitoring
Multifocal ERG / Full-field ERG	Diagnosis of AZOOR (ERG amplitude reduction even with nearly normal fundus). Monitoring of Birdshot activity (30Hz flicker delay is most sensitive). Assessment of treatment efficacy.

Disease-specific guide: “Which test first?”

When MEWDS is suspected

1. Perform FAF first (highest sensitivity; detectable even in cases where white dots are not visible).
2. FA (confirmation of wreath-like hyperfluorescence).
3. OCT (ellipsoid zone evaluation)
4. OCT-A (choriocapillaris evaluation, CNV detection)

When APMPPE is suspected

1. FA (confirmation of fluorescence reversal phenomenon)
2. ICGA (direct evaluation of choriocapillaris ischemia)
3. OCT-A (flow void evaluation)
4. If neurological symptoms are present: Brain MRI/MRA (to rule out vasculitis)

When PIC/MFC is suspected

1. OCT-A (early detection of CNV)
2. FA (evaluation of lesion leakage and CNV network)
3. ICGA (detection of subclinical lesions)
4. FAF (activity monitoring)

When serpiginous choroiditis is suspected

1. IGRA (tuberculosis exclusion is top priority)
2. ICGA (lesion activity assessment)
3. FAF (hyperautofluorescence at active margins)
4. OCT-A (choriocapillaris flow void / CNV detection)

Tips for using OCT-A

OCT-A complements conventional FA and ICGA by enabling non-invasive and repeatable diagnosis, activity assessment, CNV detection, and treatment monitoring for white dot syndromes. In particular, for PIC, it has been shown to detect CNV earlier than FA, allowing an OCTA-guided pro re nata (PRN) treatment strategy⁶⁾.

Q Which should be performed first, FA or ICGA?

A

The strategy differs by disease. In APMPPE and serpiginous choroiditis, ICGA more directly depicts choroidal circulatory disturbances, so performing ICGA simultaneously with or after FA deepens understanding of the pathology. In MEWDS, FA’s wreath-like hyperfluorescence is diagnostically important. However, since these are invasive tests, many findings can now be replaced by OCT-A, and the combination of FAF and OCT-A is used for initial evaluation^{1, 5)}.

Meaning of hypofluorescent lesions on ICGA

Hypofluorescence of white dot syndrome lesions on ICGA is a direct reflection of choroidal blood flow disruption (choriocapillaris occlusion)¹⁾. ICGA is more sensitive than FA for evaluating choroidal circulation and has the following characteristics:

• Total-phase hypofluorescence: Seen in APMPPE, serpiginous choroiditis, PIC, and MFC. Reflects severe occlusion of choriocapillaris ischemia.
• Late hypofluorescence (normal on FA): Late hypofluorescence on ICGA in MEWDS is explained by ICG uptake changes due to RPE abnormality rather than choriocapillaris involvement (choriocapillaris is generally preserved on OCT-A)¹⁾.
• Haller layer flow void → full-thickness flow void in advanced stage: A two-stage progression pattern unique to Birdshot, starting from the choroidal stroma and extending to the choriocapillaris¹⁴⁾.

ICGA is superior to FA and OCT-A in detecting clinically invisible subclinical lesions, and particularly in MEWDS and PIC, it reveals more extensive choroidal lesions than white spots^{1, 15)}.

FAF hypo- and hyperfluorescence pattern-based disease map

FAF (fundus autofluorescence) patterns reflect the metabolic state of the RPE and are useful for diagnosing white dot syndromes and assessing disease activity¹⁶⁾.

FAF pattern	Disease/stage	Meaning
High autofluorescence (hyper-AF)	MEWDS acute phase, Serpiginous active edge	Accumulation of photoreceptor degeneration products (e.g., A2E) in RPE
Low autofluorescence (hypo-AF)	APMPPE scar stage, PIC active lesion, Birdshot peripapillary	RPE loss or dysfunction
Central hypo-AF with peripheral hyper-AF halo	Serpiginous active border, PIC	RPE damage pattern at active border
Zonal abnormal AF	AZOOR	Corresponds to distribution of photoreceptor outer segment damage
Foveal granular hyperautofluorescence	MEWDS (NIR-FAF)	Noninvasive visualization of foveal granularity

In Birdshot, peripapillary confluent hypoautofluorescence is observed in 73% of cases, useful as an indicator of chronicity¹⁷⁾.

Ellipsoid zone (EZ) defect patterns on OCT

Evaluation of the ellipsoid zone (EZ, formerly the IS/OS line) plays a central role in assessing activity and predicting prognosis in white dot syndromes¹⁾.

EZ findings	Disease/stage	Prognosis
Marked EZ disruption → recovery	MEWDS acute phase → recovery phase	Good (EZ recovery linked to visual acuity recovery)
EZ loss (corresponding to lesion)	Active phase of AZOOR	No functional recovery in the lost area
EZ disruption + outer retinal hyperreflectivity	Acute phase of APMPPE	Partial residual atrophy after recovery
Sub-RPE hyperreflective elevation + EZ disruption	PIC/MFC (5-stage evolution)	Poor prognosis with CNV complication
EZ loss (with cystoid macular edema)	Birdshot advanced stage	Risk factor for poor visual prognosis

Choriocapillaris flow void on OCT-A shows high concordance with FA and ICGA findings (particularly useful in APMPPE and serpiginous choroiditis) ^{5, 13)}.

FA Fluorescence Patterns: Filling Delay vs Hyperfluorescence vs Leakage

FA Pattern	Disease	Clinical Significance
Early hypofluorescence → late hyperfluorescence (fluorescence reversal phenomenon)	APMPPE	Choriocapillaris ischemia. Early filling defect → delayed dye leakage from surrounding tissue.
Early wreath-like hyperfluorescence	MEWDS	Directly reflects RPE/photoreceptor damage. Differentiating point: does not enlarge in late phase
Early hypofluorescence → late leakage	Active serpiginous/MFC	Evidence of active choroiditis
Early arterial hyperfluorescence → late leakage	Active PIC	Suggests inflammatory CNV
Vascular leakage + optic disc hyperfluorescence (no leakage)	Birdshot	Direct evidence of retinal vasculitis
Usually normal to minimal	AZOOR	Characterized by dissociation: visual field defects and ERG abnormalities despite negative FA

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6. General Treatment

Selection of Treatment Strategy

Treatment for white dot syndromes varies greatly depending on the natural course of the disease, severity, and presence of CNV.

Observation (no treatment needed) as a principle

MEWDS, APMPPE (mild cases)

• Strong tendency for spontaneous resolution; often recovers without specific treatment²⁾
• MEWDS: White dots disappear and vision recovers within weeks. Oral steroids only when severe or with optic disc edema.
• APMPPE: White spots regress in 7–12 days; visual prognosis is usually good.

Diseases where steroid therapy is the mainstay

APMPPE (severe cases, with papillitis) · PIC (active foveal lesions) · AZOOR (severe cases)

• Start with prednisolone 30–60 mg/day and taper gradually
• If APMPPE is complicated by cerebral vasculitis, methylprednisolone pulse therapy and collaboration with neurology are required
• Severity criteria for AZOOR: corrected visual acuity in the better eye < 0.3 (Japanese Ophthalmological Society diagnostic guidelines)

Diseases requiring long-term immunosuppressive therapy

Birdshot retinochoroidopathy, serpiginous choroiditis, MFC

Birdshot (long-term management)

• Start prednisolone 0.5–1 mg/kg/day
• Mycophenolate mofetil (MMF) 2–3 g/day (first-line immunosuppressant)
• Methotrexate (MTX) 10–25 mg/week
• Azathioprine (AZA) 1–3 mg/kg/day
• Refractory cases: adalimumab (93.2% used as first-line biologic agent)
• Without treatment, 16–22% have visual acuity ≤0.1 at 10 years

Serpiginous choroiditis (long-term management)

• Prednisolone 40–80 mg/day initially (tapered)
• Azathioprine 1–2.5 mg/kg/day (first-line maintenance therapy)
• Mycophenolate mofetil / methotrexate (alternatives)
• Refractory cases: chlorambucil (most potent; ≤0.2 mg/kg/day, weekly blood tests mandatory)
• Biologics: adalimumab (91.0% recommend as first-line)
• Exclude tuberculosis (if IGRA positive, start anti-tuberculosis drugs first) before initiating immunosuppressants

Immunomodulatory therapy for MFC

Since MFC follows a chronic relapsing course, immunomodulatory drugs are often needed as maintenance therapy after steroid tapering.

Drug	Dosage guide	Notes
Methotrexate (MTX)	10–25 mg/week	Concomitant folic acid 1 mg/day. Monitor hepatotoxicity.
Azathioprine (AZA)	1–3 mg/kg/day	TPMT activity testing recommended. Monitor for myelosuppression.
Mycophenolate mofetil (MMF)	1–3 g/day	Gastrointestinal symptoms are common side effects
Cyclosporine (CsA)	3–5 mg/kg/day	Renal function and blood pressure monitoring required
Adalimumab	40 mg every 2 weeks (subcutaneous injection)	Refractory/steroid-dependent cases. Tuberculosis screening mandatory.

Management of CNV Complication

CNV is the most important complication affecting visual prognosis, and it occurs particularly frequently in PIC, MFC, and serpiginous choroiditis.

To avoid missing CNV

In PIC and MFC, some cases show CNV detected by OCT-A even when FA is negative. Sudden worsening of visual acuity or metamorphopsia is a sign of CNV, and OCTA and FAG should be performed promptly to confirm ^{6, 7)}.

Disease	CNV complication rate	Treatment
PIC	40–76%	Intravitreal anti-VEGF injection (bevacizumab, ranibizumab, aflibercept) + steroids. OCTA PRN strategy.
MFC	Up to 60%	Intravitreal anti-VEGF injection + immunomodulatory therapy
Serpiginous choroiditis	Up to 35%	Intravitreal anti-VEGF (bevacizumab, ranibizumab) injection
MEWDS/APMPPE	Rare	Anti-VEGF (if CNV confirmed)
Birdshot	Rare	Systemic immunosuppression + anti-VEGF if CNV present

Q Is anti-VEGF therapy alone sufficient for CNV?

A

Inflammatory CNV (iCNVM) differs from CNV in age-related macular degeneration; controlling the underlying inflammation is also important for preventing CNV recurrence. For PIC and MFC, a bidirectional approach with anti-VEGF and steroids (or immunosuppressants) is considered effective, and anti-VEGF monotherapy leaves a risk of recurrence^{7, 8)}.

Precautions in Treatment

Precaution	Relevant Disease
Prioritize exclusion of tuberculosis (before immunosuppression)	Serpiginous choroiditis, MFC
Consider HLA-A29 (diagnosis)	Birdshot (low sensitivity in Japanese)
Risk of intraocular pressure elevation with steroid implants	Birdshot (trabeculectomy required in up to 40%)
Bone marrow suppression and malignancy risk with chlorambucil	Serpiginous choroiditis (weekly blood tests mandatory)
Flare-up after COVID-19 infection or vaccination	PIC・MEWDS (flare-up reported)
Emergency management when cerebral vasculitis is present	APMPPE (headache, neurological symptoms → urgent brain MRI)

Q What tests should be checked first when using immunosuppressive drugs?

A

The essential tests to be checked before starting immunosuppressive drugs for white dot syndromes are as follows: ① Exclusion of tuberculosis by IGRA (QuantiFERON) (highest priority in serpiginous choroiditis), ② Viral hepatitis screening by HBs antigen, HBc antibody, and HCV antibody (to prevent reactivation due to immunosuppression), ③ Chest X-ray/CT (to exclude tuberculosis and sarcoidosis), ④ Complete blood count and liver/kidney function tests (to confirm baseline values). For biologic agents such as adalimumab, tuberculosis screening is also mandatory according to the drug package insert ^{19, 20)}.

Q How are the number and schedule of anti-VEGF therapy determined?

A

Inflammatory CNV (iCNVM) differs from age-related macular degeneration in that CNV may spontaneously regress when inflammation is controlled. Generally, a PRN (pro re nata) strategy is used, with administration each time active CNV is confirmed on OCT-A. Initial loading doses (3 consecutive injections) may be given, but combining with immunosuppressive therapy may reduce the number of required injections. Anti-VEGF alone carries a high risk of recurrence, and a bidirectional approach addressing both inflammation control and anti-VEGF is important ^{7, 8)}.

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6.5 Association with Systemic Diseases

Each disease in the white dot syndromes is known to be associated with specific systemic diseases or infections, and systematic exclusion workup before treatment is important.

Disease	Associated Systemic Disease/Condition	Clinical Significance
APMPPE	Cerebral vasculitis (MCAT), streptococcal infection, EB virus	Headache, neurological symptoms → urgent brain MRI
Birdshot	HLA-A29 (80–98% in Caucasians), similar to sarcoidosis	HLA testing aids diagnosis (low sensitivity in Japanese)
Serpiginous choroiditis	Tuberculosis (SLC) · HLA-B7/A2	IGRA positive → Anti-tuberculosis drugs first in principle
PIC	Sarcoidosis · HLA-DRB1*15	Consider chest CT and ACE measurement
MFC	IL-10 haplotype, EBV, sarcoidosis	Re-evaluate systemic workup for chronic recurrent cases
MEWDS	COVID-19 infection, post-vaccination, HLA-B51	SARS-CoV-2 infection acts as an immune trigger
AZOOR	Hashimoto’s disease, multiple sclerosis, autoimmune diseases	Consider thyroid function and autoantibody tests

Procedure for ruling out tuberculosis

Procedure for ruling out tuberculosis when serpiginous choroiditis is suspected: ① IGRA (QuantiFERON) or TST (tuberculin skin test) → ② Chest X-ray/CT → ③ If IGRA positive and active tuberculosis is ruled out, administer anti-tuberculosis drugs (isoniazid for 6 months or more) as latent tuberculosis → ④ Initiate immunosuppressive therapy after starting anti-tuberculosis drugs²⁾.

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7. Special Notes and Latest Findings for Each Disease

APMPPE and the Placoid Chorioretinitis Spectrum

APMPPE is now understood as part of the “placoid chorioretinitis spectrum” along with persistent placoid maculopathy (PPM) and relentless placoid chorioretinitis (RPC). In these three diseases, OCT-A shows a common pattern of choriocapillaris flow void, indicating that choriocapillaris ischemia is a shared pathological basis ⁵⁾.

Klufas et al. (2017) reported that OCT-A detects choriocapillaris flow void with high agreement with FA and ICGA in APMPPE, PPM, and RPC, supporting the concept of the placoid chorioretinitis spectrum ⁵⁾.

MEWDS and Foveal Granularity

Foveal granularity is a diagnostic finding seen in 74–96% of MEWDS cases and may remain as the only sign after white spots disappear. Near-infrared FAF (NIR-FAF) reveals a characteristic foveal granular pattern ⁹⁾.

Disease Spectrum of PIC and MFC

PIC and MFC (MFCwP) share a common genetic background (IL-10 haplotype, HLA-DRB1*15) and are considered different phenotypes of the same disease spectrum. The main differentiating points are the presence or absence of vitritis and anterior chamber inflammation, as well as the distribution of lesions^{1, 3)}.

Differentiating point	PIC	MFC (MFCwP)
Vitritis	Absent	Present (important differentiating point)
Anterior chamber inflammation	None	Mild
Lesion distribution	Limited to posterior pole	Posterior pole + mid-periphery
Lesion size	100–300 μm	45–350 μm
CNV complication rate	40–76%	Up to 60%

When unsure whether it is PIC or MFC

Young myopic woman with small posterior pole lesion and no vitritis → PIC is most likely. Similar lesion with vitritis and anterior chamber inflammation → MFC (MFCwP). Both diseases belong to the AZOOR complex and require long-term follow-up and regular screening for CNV.

Birdshot and HLA-A29

The association between Birdshot and HLA-A29 is one of the strongest genetic associations of any known disease, with a relative risk of 50–224 times in white patients⁴⁾. However, since HLA-A29 carriers are rare in Japanese individuals, the sensitivity of HLA-A29 positivity in diagnosis is limited. Diagnosis should emphasize the clinical findings of the SUN 2021 classification criteria (fundus findings, minimal anterior chamber inflammation, vitritis)¹⁰⁾.

AZOOR complex and viral triggers

AZOOR is thought to develop when environmental triggers such as viral infection, vaccination, or drugs are added to genetic predisposition (e.g., IL-10 haplotypes), and is understood as part of the AZOOR complex along with MEWDS, PIC, AMN, and AIBSE³⁾. Cases of MEWDS after COVID-19 infection or vaccination have been increasing worldwide, suggesting that SARS-CoV-2 may act as an immune trigger¹¹⁾.

Q Can white dot syndromes occur after COVID-19 infection or vaccination?

A

Yes. Multiple reports have described the onset or recurrence of MEWDS, PIC, and serpiginous choroiditis after COVID-19 infection, suggesting that SARS-CoV-2 infection may act as an immune trigger^{11, 12)}. For MEWDS, a systematic review of 27 cases after COVID-19 vaccination found that mRNA vaccines (Pfizer-BioNTech) were most common. Patients with a history of these conditions should consult an ophthalmologist about monitoring before and after vaccination.

Q When white dot syndrome is suspected at the first visit, what is the priority order of tests to perform?

A

The recommended priority order for initial testing when white dot syndrome is suspected is as follows: ① FAF + OCT (noninvasive, allows initial evaluation of almost all diseases; can detect high AF in MEWDS, ellipsoid zone assessment, and RPE elevation in PIC) → ② OCT-A (early detection of CNV, evaluation of choriocapillaris flow void) → ③ FA + ICGA (for definitive diagnosis and activity assessment when needed). If serpiginous choroiditis is suspected, IGRA (to rule out tuberculosis) should be prioritized before FA. For suspected AZOOR, ERG (multifocal ERG) is essential^{1, 2)}.

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8. Related Articles

急性後部多発性斑状色素上皮症（APMPPE） 両眼後極部に多発する斑状白斑と蛍光逆転現象が特徴。中枢神経血管炎合併に注意。

多発消失性白点症候群（MEWDS） 近視若年女性に好発する片眼性急性炎症。foveal granularity が診断的所見。

点状内層脈絡膜症（PIC） 若年近視女性に好発。CNV を40〜76%に合併し、OCTA による定期スクリーニングが必須。

多巣性脈絡膜炎（MFC） 硝子体炎を伴う PIC スペクトラム疾患。CNV を最大60%に合併する。

バードショット網脈絡膜症 HLA-A29 関連の慢性後部ぶどう膜炎。長期免疫抑制療法と ERG モニタリングが必須。

蛇行状脈絡膜炎 乳頭周囲から蛇行状に進展する慢性脈絡膜炎。結核との鑑別が最重要課題。

急性帯状潜在性網膜外層症（AZOOR） 眼底ほぼ正常にもかかわらず急性視野欠損・光視症をきたす外網膜症。ERG 振幅低下が診断の鍵。

特発性多巣性脈絡膜炎（IMC） MFC と連続性を持つ特発性脈絡膜炎のスペクトラム記事。

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