Whipple disease is a rare multisystem disease caused by the Tropheryma whipplei bacterium. It is most common in middle-aged white men. Migratory arthritis occurs in 80% of cases, and gastrointestinal symptoms, including diarrhea, steatorrhea, and malabsorption, occur in 75%. Intestinal loss of protein results in pitting edema and weight loss. Cardiomyopathy and valvular disease can also occur. Central nervous system involvement occurs in 10% of cases and results in seizures, dementia, and coma. Neuro-ophthalmic signs can include cranial nerve palsies, nystagmus, and ophthalmoplegia. Some patients develop a progressive supranuclear palsy–like condition (see also BCSC Section 5, Neuro-Ophthalmology, for discussion on neuro-ophthalmologic manifestations of Whipple disease).
Figure 10-16 Bilateral multifocal chorioretinitis in a patient with Whipple disease, revealed by fundus photography (left), fluorescein angiography (middle), and indocyanine green angiography (right).
(Courtesy of Wendy Smith, MD.)
Intraocular involvement is rare and occurs in less than 5% of cases. Patients can present with bilateral panuveitis and retinal vasculitis, as well as with multifocal chorioretinitis (Fig 10-16). Both anterior uveitis and moderate vitritis are present. Diffuse chorioretinal inflammation and diffuse retinal vasculitis in the perifoveal and midperipheral regions may occur. Retinal vascular occlusions and retinal hemorrhages may result from the vasculitis. Optic disc edema and, later, optic atrophy may occur. Unusual granular, crystalline deposits on the iris, capsular bag, and intraocular lens have also been reported.
Razonable RR, Pulido JS, Deziel PJ, Dev S, Salomão DR, Walker RC. Chorioretinitis and vitreitis due to Tropheryma whipplei after transplantation: case report and review. Transpl Infect Dis. 2008;10(6):413–418.
Excerpted from BCSC 2020-2021 series: Section 9 - Uveitis and Ocular Inflammation. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.