Whipple's disease was described in 1907 and was designated "intestinal lipo
dystrophy," despite the detection of bacteria in 1 specimen. This finding w
as later substantiated by the success of antibiotic therapy, which resulted
in dramatic clinical responses, and by use of electron microscopy, which d
etected monomorphic bacilli in affected tissues. Many attempts at culture f
ailed, and these bacteria were characterized as actinomycetes for the first
time by means of broad-range 16S rDNA amplification and molecular phylogen
etic methods. The name "Tropheryma whippelii" was proposed for this bacteri
um. Whipple's disease is a systemic disease that affects many organ systems
, producing protean manifestations. This article summarizes recent developm
ents with regard to this topic as well as unanswered questions regarding th
e pathogenesis and acquisition of infection, the biology and ecology of the
organism, the clinical spectrum of disease, diagnosis of the disease, and
therapy.