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In this retrospective review of 217 patients with Whipple’s disease (WD), the authors reviewed the presentations of those with ocular manifestations. Ocular signs of WD are under recognised, frequently resulting in misdiagnosis and mismanagement. Mayo Data Explorer, a Mayo Clinic data repository, was used to retrospectively identify all patients diagnosed with WD from 1 January 1980 to 10 April 2021 at Mayo Clinic, using diagnostic code variations of ICD-10: K90.81, ICD-9: 040.2, and terms ‘Whipple’s disease’ and ‘intestinal lipodystrophy’. Two-hundred and seventeen patients diagnosed with WD were identified. Inclusion criteria was listed as those with examination findings of ocular inflammatory disease, choroidal and retinal lesions, oculomotor abnormalities, or complications of ocular inflammation. Twenty-six patients with alternative diagnoses that could explain their ocular findings were excluded. Four of the 217 (1.8%) WD patients were identified as having ocular WD. All four individuals were Caucasian, including two males, two females, aged 51 to 62 years. Follow-up duration ranged from 10 to 54 months. Ocular symptoms reported were floaters and reduced vision. Two received a diagnosis of systemic WD diagnosis prior to ocular findings and two were diagnosed with WD from their ocular findings. All were diagnosed using polymerase chain reaction (PCR). The ocular manifestations included two with anterior / intermediate uveitis, one with intermediate uveitis and phlebitis, and one with chorioretinitis with vitritis. The mean duration from onset of ocular symptoms to ocular diagnosis was 11 months. All were on systemic immunomodulatory therapy prior to diagnosis. After diagnosis, three were treated with one month of IV ceftriaxone followed by one year of trimethoprim / sulfamethoxazole. Two also received IVI injections of vancomycin or ceftazidime. The fourth patient received several courses of trimethoprim / sulfamethoxazole, cefixime, rifampin, doxycycline, and finally IV ceftriaxone. The authors acknowledged the small population size and retrospective study design as limitations. The authors highlighted that WD is fatal if untreated, hence awareness of its clinical presentation is important. If WD had been considered earlier on, these patients may not have been managed with immunosuppression, potentially harmful in the context of infectious disease.

Ocular whipple disease: cases diagnosed over four decades.
Salman AR, Salomao DR, Dalvin LA, et al.
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Lucy Osborne

The University of Manchester; Royal Preston Hospital, UK.

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