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Review
. 2017 Apr;30(2):529-555.
doi: 10.1128/CMR.00033-16.

Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections

Ruben A V Dolmans  1 , C H Edwin Boel  1 , Miangela M Lacle  2 , Johannes G Kusters  3
Affiliations
Review

Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections

Ruben A V Dolmans et al. Clin Microbiol Rev. 2017 Apr.

Abstract

Whipple's disease is a rare infectious disease that can be fatal if left untreated. The disease is caused by infection with Tropheryma whipplei, a bacterium that may be more common than was initially assumed. Most patients present with nonspecific symptoms, and as routine cultivation of the bacterium is not feasible, it is difficult to diagnose this infection. On the other hand, due to the generic symptoms, infection with this bacterium is actually quite often in the differential diagnosis. The gold standard for diagnosis used to be periodic acid-Schiff (PAS) staining of duodenal biopsy specimens, but PAS staining has a poor specificity and sensitivity. The development of molecular techniques has resulted in more convenient methods for detecting T. whipplei infections, and this has greatly improved the diagnosis of this often missed infection. In addition, the molecular detection of T. whipplei has resulted in an increase in knowledge about its pathogenicity, and this review gives an overview of the new insights in epidemiology, pathogenesis, clinical manifestations, diagnosis, and treatment of Tropheryma whipplei infections.

Keywords: PCR; Tropheryma whipplei; Whipple's disease; antibiotics; clinical manifestations; histopathology; therapy.

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Figures

FIG 1
FIG 1
Schematic representation of the diagnostic algorithm. (A) Diagnostic strategy for classic Whipple's disease (WD). (B) Diagnostic strategy for chronic localized T. whipplei infection.
FIG 2
FIG 2
Microscopic detection of T. whipplei-infected duodenal mucosa. (Top) Hematoxylin-and-eosin-stained duodenal biopsy specimens with foamy macrophages in the lamina propria (arrows). The specimens were photographed with a 20× (left) and 40× (right) lens objective. (Bottom) Periodic acid-Schiff-diastase (PAS-D)-stained duodenal biopsy specimens with PAS-D-positive granules in the foamy macrophages (arrows). The same duodenal biopsy specimens as those used in the top panels were used here. The specimens were photographed with a 20× (left) and 40× (right) lens objective.
FIG 3
FIG 3
Latest proposed therapeutic strategy to treat T. whipplei infections. See the text for explanation.
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