Bilateral panuveitis in Whipple's disease: Case report

Whipple's disease is a rare disease linked to chronic infection with the intracellular gram-positive bacterium, Tropheryma whipplei. The clinical signs suggestive of this disease are the association of unexplained fever, lymphadenopathy, gastroenterological disorders (malabsorption) and inflammatory joint disorders (arthritis). However, isolated cardiological, neurological or ophthalmological forms have been described. We report the rare case of a 56- year-old patient complaining of floaters and recent visual loss, who presented with bilateral panuveitis in the absence of any systemic disorder. Clinical examination showed inflammation of the anterior segment, vitritis, inflammatory optic disc edema, focal retinitis, and venous vasculitis in both eyes. We describe the clinical characteristics and ancillary findings of the disease (fundus photos, visual field, auto-fluorescence, macular OCT, fluorescein and indocyanine green angiography). The diagnosis was made with the blood (T. whipplei) PCR test and with the help of accessory salivary gland biopsies. We describe the work-up leading to the diagnosis of Whipple's disease, the laboratory tests, and the recommended extended work-up. The patient's course was marked by complete resolution of the symptoms and clinical signs within a few months following corticosteroid therapy (1 mg/kg/day) combined with hydroxychloroquine (600 mg per day for 1 year) and life-long doxycycline therapy (200 mg per day). In conclusion, this is a rare disease which should be discussed when dealing with steroid-resistant and/or steroid-dependent chronic uveitis with a negative work-up (especially in the presence of joint and/or digestive involvement). (c) 2024 Elsevier Masson SAS. All rights are reserved, including those for text and data mining, AI training, and similar technologies.