[Whipple disease: a single or multiple origin?]

Abstract

After having been considered as an essentially digestive disease, Whipple's disease has appeared more and more to be a multivisceral disease with two main characteristics: on one hand Whipple's disease yields a diffuse infiltration of tissues by abnormal macrophages without any other inflammatory reaction; on the other hand, aspects of microbial invasion by intra or extracellular unique rod-shaped Gram+bacteria are found. This unusual pathological complex has alternatively been considered as suggestive of an immunological defect or as a very unusual type of bacterial infection. Though recent studies support the hypothesis of a primary microbial infection due to a hitherto undescribed bacterium (Tropheryma whippelii) or more or less related bacteria belonging to the actinomycetes family, they do not totally exclude a primary or acquired impairment of antigen processing by macrophages. Speculations about this fascinating pathophysiological model and about its optimal therapeutic modalities are not likely to reach a conclusion in the near future.