Tropheryma whipplei: pathogen of Whipple's disease and more

Abstract

Within the last years the understanding of infection with Tropheryma whipplei was significantly enhanced by improvement of molecular biology, microbiology and immunology. The following entities of infection or carriage, respectively, with T.whipplei must be differentiated: besides in the context of classical Whipple's disease (CWD), the rare chronic infection with T.whipplei (estimated incidence: 1:1,000,000), T.whipplei can be detected more frequently in stool specimens of children with acute gastroenteritis or asymptomatic carriers, or as a cause of isolated endocarditis. However, infection with T.whipplei only rarely results in CWD. T.whipplei was well characterized, raised in vitro and its genome completely sequenced within the last two decades. Very interesting is the resistance of the agent against glutaraldehyde.The histological detection within duodenal biopsies with "Periodic Acid Schiff" (PAS) staining still is first choice for the diagnosis of CWD. Today PCR or immunohistochemistry can identify the agent more specifically. In cases of isolated organ manifestations of e. g. joints or central nervous system the agent needs to be identified from specimen from the affected sites. Successful treatment can be achieved in most of the cases by antimicrobial therapy and first prospective treatment trials are published. However, neuronal CWD still can be progressive lethal and an immune reconstitution inflammatory syndrome (IRIS) might complicate the course of treatment and in worst case end fatal. Thus, because of the complexity of the disease a specialised reference centre should be consulted for diagnosis and treatment of CWD.