44 Infection 29 · 2001 · No. 1 © URBAN & VOGEL Infection Case Report A Case of Aortic Valve Disease Associated with Tropheryma whippelii Infection in the Absence of Other Signs of Whipple’s Disease W. Geißdörfer, I. Wittmann, G. Seitz, R. Cesnjevar, M. Röllinghoff, C. Schoerner, C. Bogdan Abstract A case of endocarditis caused by Tropheryma whippelii is reported. The 69-year-old patient was diagnosed as suffering from severe aortic regurgitation requiring aortic valve replacement, but showed no other symptoms of Whipple’s disease. T. whippelii was detected in the explanted aortic valve by broad-range PCR amplification of the 16S rDNA and subsequent sequence analysis of the product. The etiologic agent was classified as a type 2A sequence variant based on the 16S-23S intergenic spacer and the 23S rDNA (domain III) sequences. The histological examination of the aortic valve was compatible with Whipple’s disease. A duodenal biopsy revealed an infection with Giardia lamblia, but T. whippelii and histological signs of Whipple’s disease were not detectable. Key Words Tropheryma whippelii · Whipple’s disease · Endocarditis · PCR Infection 2001; 29: 44–47 DOI 10.1007/s15010-001-0135-9 Introduction Whipple’s disease is a rare systemic bacterial infection re- sulting in diarrhea, weight loss, fever, lymphadenopathy and migratory polyarthritis [1]. In addition, cardiac manifesta- tions or involvement of the central nervous system (CNS) and/or the eyes have been observed, which usually devel- oped in parallel to the typical gastrointestinal symptoms of the disease [1–3]. Even in the absence of overt neurologi- cal symptoms, CNS involvement is common in Whipple’s disease. Therefore, only antibiotics which cross the blood- brain barrier (e.g. co-trimoxazole, ceftriaxone, cefixime and rifampicin) should be used for treatment [1]. The etiologic agent of Whipple’s disease was classified as Tropheryma whippelii on the basis of its 16S rDNA se- quence. This gram-positive bacterial species is related to actinobacteria [4] and cellulomonads [5]. To date, cultiva- tion of the bacterium is still difficult and was achieved only twice, i.e. in human blood monocytes [6] and in human fi- broblasts [7]. Therefore, Whipple’s disease is usually diag- nosed by the histopathological analysis of duodenal biopsy specimens, in which CD68 + macrophages loaded with cy- toplasmic periodic acid Schiff (PAS)-positive,sickle-formed inclusions are found in the lamina propria and the submu- cosa. It is important to note that in the absence of gas- trointestinal symptoms (diarrhoea, weight loss), a negative histological result does not exclude the diagnosis of Whip- ple’s disease [8].To increase the sensitivity of the diagnos- tic procedures various protocols for PCR have been de- signed which target the 16S rDNA [4, 9], the 16S-23S rDNA spacer [10, 11] or the domain III of the 23S rDNA [12]. Se- quence analyses of the amplification products identified the T. whippelii variants 1 to 6 (16S-23S rDNA spacer) as well as A and B (23S rDNA), which may be relevant to our un- derstanding of the epidemiology of Whipple’s disease [10–12]. So far, these sequence variants do not seem to lead to different clinical manifestations [11] or to vary in their regional distribution [10]. Here, we report on a patient with an incompetent aortic valve which was found to be infected with T. whippelii in the absence of any other clinical sign of Whipple’s disease. Case Report In 1995, the 69-year-old male patient, who was known to have type II diabetes mellitus, high blood pressure and an intermittent in- crease of the uric acid serum levels with no definitive history of gout, had a posterior myocardial infarction which was treated by percutaneous transluminal coronary angioplasty of the Ramus cir- cumflexus of the left coronary artery.At that time aortic valve re- gurgitation was first diagnosed. In September 1999, the patient had W. Geißdörfer (corresponding author), I Wittmann, M. Röllinghoff, C. Schoerner, C. Bogdan Institute of Clinical Microbiology, Immunology and Hygiene, Friedrich- Alexander University of Erlangen-Nürnberg,Wasserturmstr. 3, D-91054 Erlangen, Germany; Phone: (+49/9131) 85-25744, Fax: -1001, e-mail: walter.geissdoerfer@mikrobio.med.uni-erlangen.de G. Seitz Institute of Pathology, Bamberg Clinic, D-96049 Bamberg, Germany R. Cesnjevar Center for Cardiac Surgery; Friedrich-Alexander University of Erlangen- Nürnberg, D-91054 Erlangen, Germany Received: September 6, 2000 • Revision accepted: December 3, 2000