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Whipple Endocarditis without Overt Gastrointestinal Disease: Report of Four Cases

Jacques G.H. Gubler, MD; Marcel Kuster, MD; Fabrizio Dutly, PhD; Fridolin Bannwart, MD; Martin Krause, MD; Hans Peter Vögelin, MD; Gianni Garzoli, MD; and Martin Altwegg, PhD
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From Stadtspital Triemli and University of Zürich, Zürich, Switzerland; Kantonsspital, Münsterlingen, Switzerland; Kreisspital, Bülach, Switzerland; and Schwerpunktspital Zimmerberg, Wädenswil, Switzerland.

Acknowledgments: The authors thank R. Weber, Division of Infectious Diseases, and members of the Department of Pathology, University Hospital, Zürich, who participated in the care of patient 2; the members of the Division of Cardiosurgery, University Hospital, Zürich, who operated on patients 1, 2, and 4; and A. von Graevenitz, who read the manuscript.

Grant Support: Swiss National Science Foundation (32-50790.97) (Dr. Altwegg).

Requests for Reprints: Jacques G.H. Gubler, MD, Department of Medicine, Stadtspital Triemli, Birmensdorferstrasse 497, CH-8063 Zürich, Switzerland.

Current Author Addresses: Drs. Gubler and Kuster: Department of Medicine, Stadtspital Triemli, CH-8063 Zürich, Switzerland.

Drs. Dutly and Altwegg: Department of Medical Microbiology, University of Zürich, Gloriastrasse 30, CH-8028 Zürich, Switzerland.

Dr. Bannwart: Department of Pathology, Stadtspital Triemli, CH-8063 Zürich, Switzerland.

Dr. Krause: Department of Medicine, Kantonsspital, CH-8596 Münsterlingen, Switzerland.

Dr. Vögelin: Department of Medicine, Kreisspital, 8180 Bülach, Switzerland.

Dr. Garzoli: Schwerpunktspital Zimmerberg, Schlossbergstrasse 32, 8820 Wädenswil, Switzerland.

Ann Intern Med. 1999;131(2):112-116. doi:10.7326/0003-4819-131-2-199907200-00007
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Background: Cardiac manifestations of Whipple disease are rarely diagnosed before death.

Objective: To describe four patients with endocarditis caused by Tropheryma whippelii who did not have overt gastrointestinal disease.

Design: Case series.

Setting: Five hospitals in eastern Switzerland.

Patients: Three men and one woman undergoing replacement of insufficient heart valves.

Measurements: Histologic characteristics of heart valves and intestinal biopsy; broad-range and specific polymerase chain reaction for T. whippelii.

Results: Tropheryma whippelii was found in the heart valves (three aortic valves and one mitral valve) of four patients with culture-negative endocarditis necessitating valve replacement. All patients had arthralgia for different lengths of time. Only one patient had mild gastrointestinal symptoms. Histologic characteristics of intestinal mucosa were normal in all patients, and polymerase chain reaction on intestinal biopsy was positive for T. whippelii in only one patient, who did not have diarrhea. In all patients, arthralgia resolved promptly after institution of antibiotic therapy. Disease did not recur in any patient after prolonged antibiotic therapy with cotrimoxazole.

Conclusion: In patients with culture-negative endocarditis, the absence of clinical, microscopic, or microbiological evidence of gastrointestinal disease did not rule out T. whippelii.


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Microscopy of aortic heart valve withTropheryma whippelii-endocarditis.A.B.C.D.

All photographs show histologic views of the aortic valve of patient 1. Macroscopic view. Thickened sclerotic and calcified valve with chronic vegetations and focal ulceration (van Gieson-Elastin; original magnification, ×3). Fibrinoleukocytic vegetation on the valve near the annular portion (van Gieson-Elastin; original magnification, ×25). Periodic acid-Schiff-positive inclusions in presumed necrotic macrophages, surrounded by fibrin and several leukocytes (periodic acid-Schiff; original magnification, ×100). Abundant rod-shaped bacteria, dispersed and in clumps (Warthin-Starry silver impregnation; original magnification, ×630).

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