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Whipple’s disease 

Whipple’s disease

Chapter:
Whipple’s disease
Author(s):

H.J.F. Hodgson

DOI:
10.1093/med/9780199204854.003.151006_update_001

Update:

Clinical and diagnostic features; further reading added.

Updated on 28 Nov 2012. The previous version of this content can be found here.
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date: 27 March 2017

Whipple’s disease is an uncommon infection caused by the actinomycete Tropheryma whipplei. It is most commonly diagnosed when overt small-intestinal disease leads to malabsorption, but before this there are often several years of nonspecific prodromal manifestations such as fever and arthralgia/arthritis, and the disease can present in many other ways, e.g neurological manifestations (of many forms, including movement disorders, ocular manifestations and meningitis) or endocarditis.

Diagnosis usually depends upon (1) demonstration of classical histological features in the small intestine, which is thick and oedematous, with stubby or absent villi and dilated lacteals, and with the lamina propria stuffed with macrophages containing foamy material which stains brilliant magenta with periodic acid–Schiff reagent (diastase resistant); and (2) positive identification of Tropheryma whipplei DNA by polymerase chain reaction. Molecular techniques have increased the frequency with which individuals with minimal or absent gastrointestinal disease are diagnosed.

Treatment is with antibiotics (initially a bactericidal agent with CSF penetration, followed by long-term therapy with e.g. trimethoprine-sulphamethiazole). Clinical improvement occurs within a few weeks, but prolonged treatment for at least a year is recommended. Relapse can occur, even after many years, especially when progressive central nervous system disease occurs in the absence of other systemic manifestations.

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