© Springer International Publishing AG 2017
George N. Papaliodis (ed.)Uveitis10.1007/978-3-319-09126-6_1616. Whipple’s Disease
(1)
Department of Ophthalmology, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, MA 02114, USA
Keywords
WhippleUveitis Tropheryma whippelii Introduction
Whipple’s disease is a rare systemic disorder caused by the Gram positive bacterium Tropheryma whippelii (T. whippelii). Although the initial report was characterized by a malabsorption syndrome involving the small intestine, this malady can affect the joints, central nervous system, cardiovascular system, and eyes. The ocular manifestations are similarly uncommon (approximately 5 % of patients with Whipple’s disease will have ocular involvement) but can include vitritis, retinal vasculitis, choroiditis, ophthalmoplegia, and keratitis [1].
Epidemiology
The first case report of this entity in the medical literature was in 1895, but this was not recognized as a specific disease until George Hoyt Whipple described a patient with diarrhea secondary to malabsorption, weight loss, migratory polyarthritis, and mesenteric lymphadenopathy in 1907 [2]. The disorder is exceptionally rare with fewer than 1000 cases reported world-wide. The condition appears to be associated with human leukocyte antigen HLA-B27 haplotype [3] and is more common in white males (male to female ratio approximately 8–9:1).