Tropheryma whipplei endocarditis

Abstract

Tropheryma whipplei endocarditis differs from classic Whipple disease, which primarily affects the gastrointestinal system. We diagnosed 28 cases of T. whipplei endocarditis in Marseille, France, and compared them with cases reported in the literature. Specimens were analyzed mostly by molecular and histologic techniques. Duke criteria were ineffective for diagnosis before heart valve analysis. The disease occurred in men 40-80 years of age, of whom 21 (75%) had arthralgia (75%); 9 (32%) had valvular disease and 11 (39%) had fever. Clinical manifestations were predominantly cardiologic. Treatment with doxycycline and hydroxychloroquine for at least 12 months was successful. The cases we diagnosed differed from those reported from Germany, in which arthralgias were less common and previous valve lesions more common. A strong geographic specificity for this disease is found mainly in eastern-central France, Switzerland, and Germany. T. whipplei endocarditis is an emerging clinical entity observed in middle-aged and older men with arthralgia.

Keywords: Tropheryma whipplei; Whipple disease; Whipple’s disease; arthralgia; bacteria; endocarditis.

Figure 1

Number of reported cases of Tropheryma whipplei endocarditis per 1 million inhabitants in each area of France over 10 years. Data from this series and the literature (–24) were included. Among the metropolitan areas in France, the incidence of T. whipplei endocarditis is significantly more frequent in the Rhône-Alpes area than in 11 others areas (Alsace, Aquitaine, Basse-Normandie, Bourgogne, Centre, Champagne-Ardenne, Haute-Normandie, Ile de France, Languedoc-Roussillon, Midi-Pyrénées, and Nord Pas-de-Calais; p = 0.04, p = 0.004, p = 0.048, p = 0.04, p = 0.01, p = 0.04, p = 0.02, p<0.001, p = 0.04, p = 0.007, p = 0.006, respectively). The incidence rate is also significantly more frequent in the Pays de la Loire area than in 6 other areas (Aquitaine, Bretagne, Centre, Ile-de France, Lorraine, Midi-Pyrénées, Nord Pas de Calais; p = 0.04, p = 0.04, p = 0.04, p = 0.003, p = 0.03, p = 0.02, respectively).

Figure 2

Aortic valve from patient with Tropheryma whipplei endocarditis. A) Hematoxylin–eosin–saffron stain (original magnification ×100). B) Foamy macrophages containing characteristic inclusion bodies (periodic acid–Schiff stain; original magnification ×200). C) Immunostaining of T. whipplei with polyclonal rabbit antibody against T. whipplei and Mayer’s hemalum counterstain (original magnification ×100). No destruction of this valve is visible.

Figure 3

Number of reported cases of Tropheryma whipplei endocarditis per 1 million inhabitants in each country of Europe (www.statistiques-mondiales.com/union_europeenne.htm).

Figure 4

Number of cases of Tropheryma whipplei endocarditis reported in the literature since the first detection of this condition in 1997. Cases in 2013 are reported through April.