History of present illness
We present a case of a 58-year-old woman referred for treatment-refractory anterior uveitis and vitritis of her left eye after cataract surgery 2 years earlier. She also reported inflammatory arthritis (seronegative) and abdominal pain with nausea and vomiting for over 10 years. She was HLA-B27 positive. Subjectively, she reported persistent, symptomatic floaters in the left eye with blurry vision. Previously, she was managed with topical and intravitreal corticosteroids and systemic immune suppression.
Ocular examination findings
Visual acuity with pinhole correction was 20/20 in the right eye and 20/30 in the left eye. Intraocular pressures were normal. External and anterior segment examinations were normal in the right eye. There were 1+ anterior chamber cells of the pseudophakic left eye along with diffuse vitreous cells, but no chorioretinal lesions or vitreous opacities were present.
Imaging
Optical coherence tomography (OCT) of the left eye showed irregular macular thickening, an epiretinal membrane with flattening of the foveal depression and inner retinal striae, and no subretinal deposits or intraretinal fluid. The choroid had normal thickness.
Questions to ask
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Was the cataract surgery complicated? Certain complications increase the risk of postoperative indolent endophthalmitis.
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No
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Has the patient experienced neurological symptoms, fevers, chills, or night sweats? Vitreoretinal lymphoma should be considered in the differential diagnosis for treatment-refractory uveitis and frequently has concurrent central nervous system lesions.
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No
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Has the patient had a high-risk infectious exposure? Multiple infectious etiologies may present with chronic uveitis, including tuberculosis and Lyme disease.
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No
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Has the patient experienced weight loss from her gastrointestinal symptoms? Whipple disease affects gastrointestinal absorption, leading to diarrhea and potential weight loss.
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Yes
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Assessment
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A 58-year-old woman with a history of seronegative inflammatory arthritis and GI symptoms is referred for treatment of a refractory anterior uveitis and vitritis in her left eye after cataract surgery 2 years earlier. Examination revealed left eye anterior chamber cells, vitreous cells, and an epiretinal membrane with inner retinal striae.
Differential diagnosis
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Chronic endophthalmitis
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Vitreoretinal lymphoma
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Sarcoidosis
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Mycobacterium avium-intracellulare complex
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Tuberculosis
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Presumed ocular histoplasmosis
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Multifocal choroiditis and panuveitis—no chorioretinal lesions were present in this patient
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Amyloidosis—vitreous does not show glass wool opacities, and no material is seen emanating from the retinal vessels
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Lyme disease
Working diagnosis
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Ocular Whipple disease
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Whipple disease can present with seronegative inflammatory arthritis, diarrhea, weight loss, chronic anterior uveitis, vitritis, and typically follows an indolent course.
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Examination/multimodal testing and results
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Slit lamp examination
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The left eye had 1+ anterior chamber cells, diffuse vitreous cells, and 1+ vitreous haze. Other possible findings may include corneal keratic precipitates, crystalline keratopathy, and iris or lens deposits.
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Fundus photographs
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There was an epiretinal membrane with traction on the retina. Patients may also exhibit vitreous infiltrates, chorioretinal lesions, periphlebitis, macular edema, optic disc edema, and optic atrophy.
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Neuroophthalmic examination
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Neuroophthalmic findings were not noted. Oculomasticatory myorhythmia, which involves a smooth pendular convergent-divergent nystagmus with concurrent contractions of the masticatory muscles, is considered a pathognomonic sign of Whipple disease.
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OCT
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An OCT of the left eye showed macular thickening, an epiretinal membrane, flattening of the foveal depression, and inner retinal striae without cystoid macular edema.
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Vitreous biopsy
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The patient had a therapeutic and diagnostic pars plana vitrectomy. The specimen demonstrated the classic finding of foamy histiocytes with intracytoplasmic, PAS+ gram-positive rods consistent with Tropheryma whipplei ( Fig. 40.1 ). T. whipplei was then confirmed in the specimen by polymerase chain reaction (PCR).
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