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Abstract
The characteristic features of Whipple's disease include abdominal pain, diarrhoea,
wasting, and arthralgias, with the causative agent, Tropheryma whipplei, being detected
mainly in intestinal biopsies. PCR technology has led to the identification of T.
whipplei in specimens from various other locations, including the central nervous
system and the heart. T. whipplei is now recognized as one of the causes of culture-negative
endocarditis, and endocarditis can be the only manifestation of the infection with
T. whipplei. Although it is considered a rare disease, the true incidence of endocarditis
due to T. whipplei is not clearly established. With the increasing use of molecular
methods, it is likely that T. whipplei will be more frequently identified. Questions
also remain about the genetic variability of T. whipplei strains, optimal diagnostic
procedures and therapeutic options. In the present study, we provide clinical data
on four new patients with documented endocarditis due to T. whipplei in the context
of the available published literature. There was no clinical involvement of the gastrointestinal
tract. Genetic analysis of the T. whipplei strains with DNA isolated from the excised
heart valves revealed little to no genetic variability. In a selected case, we describe
acridine orange staining for early detection of the disease, prompting early adaptation
of the antibiotic therapy. We provide long-term follow-up data on the patients. In
our hands, an initial 2-week course of intravenous antibiotics followed by cotrimoxazole
for at least 1 year was a suitable treatment option for T. whipplei endocarditis.