[Whipple's disease]

Abstract

For many years, Whipple's disease was considered a rare, mainly intestinal disease causing malabsorption. At present, however, it appears to be multivisceral mainly occuring in subjects with specific and subtle cell-mediated immunity defects. Until recently, diagnosis and follow-up of treatment efficacy depended on PAS positive macrophage inclusions in duodenal biopsies. New diagnostic methods based on PCR gene amplification and immunohistochemistry are now available by DNA sequencing and culture, respectively, of the causal bacteria, which was recently renamed Tropheryma whipplei. Although results are still empirical, and the first randomized study is in progress, an evolution in the choice and duration of antibiotic treatment of this normally fatal disease has led to a marked reduction in clinical relapses, especially for neurological manifestations. The present review shows how recent medical advances have completely transformed the understanding of a disease first described a century ago.