Tropheryma whipplei Endocarditis - Vol. 19 No. 11 - November 2013 - Emerging Infectious Disease journal - CDC
Volume 19, Number 11—November 2013
Tropheryma whipplei Endocarditis
Whipple disease was first described in 1907 (1). This chronic infection is characterized by histologic indication of gastrointestinal involvement, determined by a positive periodic acid–Schiff (PAS) reaction in macrophages from a small bowel biopsy sample (2). It is caused by Tropheryma whipplei and encompasses asymptomatic carriage of the organism to a wide spectrum of clinical pathologic conditions, including acute and chronic infections (1,2).
AbstractTropheryma 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.
In 1997, T. whipplei was first implicated as an agent of blood culture–negative endocarditis in 1 patient by use of broad-range PCR amplification and direct sequencing of 16S rRNA applied to heart valves from patients in Switzerland (3). Two years later, 4 additional cases were reported in Switzerland (4). In 2000, the first strain of T. whipplei was obtained from the aortic valve of a patient with blood culture–negative endocarditis (5).
Blood culture–negative endocarditis accounts for 2.5%–31.0% of all cases of endocarditis. The incidence rate of T. whipplei endocarditis among blood culture–negative endocarditis cases has not been established; however, at our center (Assistance Publique Hôpitaux de Marseille, Marseille, France), this incidence rate was estimated to be 2.6% (6). In Germany, the reported incidence rate for T. whipplei endocarditis is 6.3%: T. whipplei was the fourth most frequent pathogen found among 255 cases of endocarditis with an etiologic diagnosis and was the most common pathogen associated with blood culture–negative endocarditis. This incidence rate exceeds rates of infections caused by Bartonella quintana; Coxiella burnetii; and members of the Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella spp. group (7). Smaller studies found incidence rates of 3.5% in Denmark (8), 4.3% in Switzerland (9), 7.1% in the Czech Republic (10), 2.8% in Spain (11), and none in Algeria (12). We describe 28 cases of T. whipplei endocarditis and compare them with cases reported in the literature.