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Noninfectious Inflammatory Diseases

MULTIFOCAL CHOROIDITIS

Rebecca R. Soares, MD, MPH and Sonia Mehta, MD

• Characterized by multifocal chorioretinal inflammatory lesions that leave pigmented fibrotic scars; typically bilateral and not associated with any systemic disease
  
• Usually occurs in young (~30 years old) Caucasian females with myopia
  
• Course is typically chronic with recurrent inflammation
  
• Visual acuity preserved in majority of patients (~65% to 75% retain ≥ 20/40 or better)
  
• Most common cause of decreased vision is choroidal neovascularization (CNV). Other causes include macular edema, foveal scarring, and epiretinal membrane (ERM).

Bilateral yellow-white punched out chorioretinal lesions (size 50 to 200 μm) with variable pigmented fibrotic scars in posterior pole and periphery often clustered nasal to disc (Figures 4-1A, 4-1B, and 4-2A); Schlaegel lines: peripheral curvilinear streaks of chorioretinitis; vitritis and anterior chamber inflammation in multifocal choroiditis with panuveitis (MFCPU); other findings: peripapillary atrophy, disc edema, macular edema, retinal detachment, CNV, ERM

Testing

• No diagnostic laboratory test for multifocal choroiditis. Testing may be done to rule out infectious or noninfectious causes. Tuberculosis: QuantiFERON-TB Gold or purified protein derivative; syphilis: rapid plasma reagin (RPR) and fluorescent treponemal antibody absorption (FTA-ABS); sarcoidosis: chest x-ray and angiotensin converting enzyme (ACE); histoplasmosis: urine anion gap and/or serum Ab.
  
• Fluorescein angiography (FA): early hypofluorescence of lesions followed by late staining; early hyperfluorescence with late leakage if CNV present (Figures 4-1C and 4-1D)
  
• Indocyanine green (ICG) angiography (ICGA): early and late hypocyanescence of lesions
  
• Fundus autofluorescence: active lesions—hyperautofluorescence of retinal pigment epithelial (RPE) elevations, central absent autofluorescence if dehiscence of RPE; inactive lesions—absent autofluorescence in areas of RPE scarring or atrophy (Figure 4-2B)
  
• Optical coherence tomography (OCT): active lesions—RPE elevation with sub-RPE infiltration of homogenous debris and outer retinal changes; CNV—heterogenous subretinal/sub-RPE material, subretinal fluid, intraretinal edema; CME—presence is one indicator of active disease; enhanced depth imaging—increased choroidal thickening in areas corresponding to RPE elevation (Figure 4-2C)

POSTERIOR SCLERITIS

Matthew Trese, DO, MA and Thomas J. Wubben, MD, PhD

• Rarest form of scleritis and is likely under recognized given significant variability in its clinical presentation. Underlying etiology often idiopathic but there are strong associations with autoimmune and infectious conditions in approximately half of cases.
  
• Female predominance, primarily unilateral, and may be associated with anterior scleritis

Signs and Symptoms

Periocular pain, blurred vision, headache, photophobia

Exam Findings

Anterior segment may appear normal (unless there is concomitant anterior scleritis), proptosis, or angle closure glaucoma secondary to uveal effusion; posterior segment may have retinal and/or choroidal folds, serous retinal detachment, macular edema, optic disc edema, and subretinal mass due to nodular posterior scleritis

Testing

• Laboratory evaluation is to rule out associated autoimmune and infectious conditions: complete blood count (CBC), rheumatoid factor, anti-CCP, anti-nuclear antibody, antineutrophil cytoplasmic antibodies, ACE, lysozyme, RPR/FTA, QuantiFERON-TB Gold
  
• Imaging studies may include the following:
  
  
  
  • B-scan ultrasound: posterior scleral thickening (> 2 mm is abnormal) and/or a “T-sign” showing fluid in sub-Tenon’s space (Figure 4-3)
    
  • Computed tomography or magnetic resonance imaging with contrast: enhancement and thickening of posterior sclera, classically described as a “ring sign” on coronal sections through globe

• Posterior scleritis poses a significant threat to vision and often mandates more aggressive therapy than anterior scleritis.
  
• If a systemic disease can be identified, targeted therapy against the infectious or inflammatory process is warranted.
  
• First-line treatment: oral nonsteroidal anti-inflammatory drugs are generally insufficient; prednisone (1 mg/kg once a day or 60 mg once a day) with gradual tapering depending on clinical response is often required
  
• Second-line treatment: immunomodulatory therapy such as antimetabolites, alkylating agents or T-cell inhibitors, administered in conjunction with a rheumatologist
  
• Third-line treatment: biologic agents targeted against tumor necrosis factor alpha (TNF-α) and cluster of differentiation-20 (CD-20) have been used if refractory to systemic steroids and conventional immunomodulatory therapy

MULTIPLE EVANESCENT WHITE DOT SYNDROME

Paul S. Baker, MD

• Acute, unilateral, inflammatory disease characterized by multiple small, white dots in deep retina and retinal pigment epithelium (Figure 4-4A)
  
• Etiology is unknown, but viral origin and genetic predisposition have been suggested
  
• Strong female predominance (~75%) and usually affects young adults

Signs and Symptoms

Mild to moderate acute vision loss, usually unilateral; central or temporal scotoma, photopsias; may be preceded by a recent viral flu-like illness

Exam Findings

White dots are concentrated in paramacular area, sparing fovea, which usually has granular appearance with yellowish-orange specks (peau d’orange); vitreous cells, retinal venous sheathing; optic disc edema

• MEWDS is a clinical diagnosis; there is no diagnostic laboratory testing.
  
• Fundus autofluorescence: lesions show characteristic hyperautofluorescence which may be more numerous than clinically apparent (Figure 4-4B)
  
• FA: Early hyperfluorescent spots in a wreath-like configuration with late staining. Optic disc and vascular leakage is often present (Figures 4-4C and 4-4D).
  
• ICG: more numerous hypocyanescent lesions than expected based on clinical exam; peripapillary hypocyanescence
  
• OCT: subtle disruption of photoreceptor layer
  
• Electroretinogram (ERG): reduced a-wave and early receptor potential (ERP) amplitudes
  
• Visual field testing: enlarged blind spot

ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY

David Xu, MD and Sonia Mehta, MD

• Rare, idiopathic, bilateral inflammatory condition that usually affects young patients 20 to 40 years old with equal sex predilection
  
• One-third experience a flu-like prodrome
  
• Uncommonly associated with central nervous system (CNS) vasculitis or meningoencephalitis

• Acute: mild vitritis; multifocal creamy yellow-white placoid retinal lesions in the posterior pole to mid-periphery at the level of the RPE and choroid (Figure 4-5)
  
• Chronic: lesions usually fade over 1 to 2 weeks and new lesions may develop; may leave patches of variably pigmented RPE atrophy

Testing

• FA: early hypofluorescence of lesions followed by late staining (Figures 4-6A and 4-6B)
  
• ICG angiography: early and late hypocyanescence of lesions; more lesions are seen on ICG than clinically apparent (see Figures 4-6A and 4-6B)
  
• Fundus autofluorescence: hyperautofluorescence of lesions
  
• OCT: lesions show hyperreflectivity from ellipsoid zone to outer plexiform layer (Figure 4-7)
  
• OCT angiography: areas of flow deficit in the choriocapillaris corresponding to the placoid lesions

Differential Diagnosis

MEWDS, serpiginous choroidopathy, relentless placoid choroiditis, multifocal choroiditis, Vogt-Koyanagi-Harada syndrome, syphilitic chorioretinitis, tuberculosis, fungal disease, choroidal metastasis, lymphoma

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