History of present illness
We present a case of a 38-year-old Parisian man with an unremarkable past medical history referred for management of multifocal choroiditis (MFC). While in Paris in 2011, he experienced sudden-onset scotomas bilaterally with a prodrome of headaches without neck pain. He denied fevers, redness, pain, photophobia, photopsia, metamorphopsia, or floaters. He underwent two oral steroid tapers and was transitioned to azathioprine, which was stopped because of drug-induced pancreatitis. Our patient was then started on mycophenolate mofetil.
Ocular examination findings
Visual acuity without correction was 20/20 in both eyes. Intraocular pressure was normal. External and anterior segment examination was unremarkable. Dilated fundus examination showed bilateral peripapillary atrophy with punctate scars surrounding the optic disc; pigmented, punched-out confluent scars within the macula but sparing the fovea; and prominent choroidal pattern, MFC-type scars in the posterior pole and midperiphery without vitreous cell or haze ( Fig. 30.1 A and B).
Previous testing
Our patient had elevated angiotensin-converting enzyme levels with no hilar lymphadenopathy noted on computed tomography of the lung. Biopsy of the accessory salivary gland was normal. Other normal/negative tests include lumbar puncture, antineutrophilic cytoplasmic antibody, antinuclear antibodies, and extensive infectious disease workup.
Questions to ask
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Is the patient from a region endemic to histoplasmosis, or has he had exposure to bats (e.g., while spelunking)? The lesions in MFC can resemble presumed ocular histoplasmosis syndrome. In fact, the term MFC was initially used to describe eyes with presumed ocular histoplasmosis syndrome (POHS).
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No
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Are the fundus lesions confined to the posterior pole? It can be difficult to distinguish punctate inner choroidopathy (PIC) and MFC, but patients with PIC are typically myopic women with smaller lesions confined to the posterior pole without associated intraocular inflammation.
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No, the lesions are large, confluent, and present in the posterior pole as well as the midperiphery without intraocular inflammation.
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Did the patient have any recent travel history before the onset of symptoms? This information is helpful for an infectious workup, including tuberculosis (TB), toxoplasmosis, and West Nile virus.
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The patient traveled to Vietnam shortly before the onset of his symptoms, but extensive infectious disease workup, including purified protein derivative skin testing and QuantiFERON-TB Gold, was negative.
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Does the patient have a history of cancer? Malignancies have been reported as masquerade syndromes of MFC.
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No
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Assessment
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This is a case of a 38-year-old man with no past medical history demonstrating bilateral idiopathic MFC.
Differential diagnosis
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Infectious
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TB
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POHS
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Syphilis
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Pneumocystis choroiditis
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West Nile virus
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Recurrent toxoplasmosis
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Noninfectious
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Sarcoidosis
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Birdshot chorioretinopathy (can resemble active MFC)
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Acute posterior multifocal placoid pigment epitheliopathy with progression to relentless placoid chorioretinopathy
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PIC
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Malignant
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Lymphoma
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Metastasis
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Working diagnosis
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Idiopathic MFC
Multimodal testing and results
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Fundus photographs
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Fundus examination showed bilateral peripapillary atrophy with adjacent scarring, punched-out pigmented scars within the macula (following blood vessels) with sparring of the fovea, and prominent choroidal pattern, MFC-type scars of varying sizes in the posterior pole and midperiphery ( Fig. 30.1 A, B).
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Active lesions are typically yellow-white because of choroidal lesions with blurred margins due to overlying retinal edema, whereas inactive lesions are gray with more defined borders. Punctate hemorrhages can occasionally be visualized.
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Fundus autofluorescence
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Left eye showed an area of hyperautofluorescence within a scar at the termination of the superotemporal arcade; right eye did not show hyperautofluorescence within the fovea ( Fig. 30.1 C, D).
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In active disease, diffuse hyperautofluorescent lesions can be seen in the peripapillary region and posterior pole with late staining of the optic nerve. As the inflammation subsides, the lesions become atrophic and hypoautofluorescent.
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Optical coherence tomography (OCT)
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OCT showed foveal sparing scars with focal loss of outer retina in the right eye and foveal sparing scars with focal loss of outer and inner retina in the left eye ( Fig. 30.2 ).
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