Tropheryma whipplei: Erreger verschiedener Infektionen und Pathogen des Morbus Whipple

Zusammenfassung

In den letzten Jahren haben Fortschritte in der Molekularbiologie, Mikrobiologie und Immunologie das Verständnis der Infektion mit Tropheryma whipplei entscheidend verbessert. Eine Infektion mit T. whipplei führt nur selten zum klassischen M. Whipple. Man sollte folgende Entitäten der Infektion bzw. Besiedelung mit T. whipplei unterscheiden: Neben dem klassischen Morbus Whipple, der seltenen chronischen Infektion mit T. whipplei (geschätzte Inzidenz: 1 : 1.000.000), wird T. whipplei sehr viel häufiger im Stuhl von Kindern mit akuter Gastroenteritis, von symptomlosen Ausscheidern oder als Ursache für isolierte Endokarditis nachgewiesen. T. whipplei wurde in den letzten zwei Jahrzehnten besser charakterisiert, durch Einsatz spezieller Kulturtechniken angezüchtet, und sein Genom wurde komplett sequenziert. Interessant ist die Resistenz des Erregers gegen Glutaraldehyd. Der histologische Nachweis mittels PAS-Färbung („Periodic Acid Schiff“) in Dünndarmbiopsien ist nach wie vor die Methode der Wahl zur Diagnose des klassischen M. Whipple. Heute kann mittels PCR oder Immunhistochemie der Erreger spezifischer nachgewiesen werden. Bei isoliertem Organbefall z. B. der Gelenke oder des Zentralnervensystems muss zum Erregernachweis Probenmaterial aus der betroffenen Region herangezogen werden. Die Behandlung ist in den meisten Fällen durch Antibiotika erfolgreich möglich und erste prospektive Behandlungsstudien wurden abgeschlossen. Dennoch kann der neuronale M. Whipple auch heute noch progredient letal verlaufen. Ein Immunrekonstitutionssyndrom (IRIS) kann den Verlauf der Therapie komplizieren und im schlimmsten Fall fatal enden. Daher sollte auf Grund der Komplexität für die Diagnostik und die Therapie des klassischen M. Whipple immer ein spezialisiertes Zentrum konsultiert werden.

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.

Schlüsselwörter

Morbus Whipple - Tropheryma whipplei - chronische Diarrhö - Arthritis - Endokarditis - inflammatorisches Immunrekonstitutionssyndrom (IRIS)

Keywords

Whipple’s disease - Tropheryma whipplei - chronic diarrhea - arthritis - endocarditis, immune reconstitution inflammatory syndrome (IRIS)

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