Tropheryma whippleiEndocarditis

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.

Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections

“…However, one should be aware that PAS staining has a poor specificity in brain biopsy samples (130,177). Depending on the localization of the infection, T. whipplei can also be found in bone marrow, lymph nodes, skin, liver, muscle, eye, and lung (10,62,103,113,120,137,178). It is important to realize that in chronic localized infections, PAS-stained duodenal biopsy specimens may be negative (102,130,162,179,180).…”

“…In most patients, there are no other manifestations of classic Whipple's disease (113,(149)(150)(151)(152), and clinical signs are similar to those of cardiac disease with negative blood cultures (113). In spite of the negative blood cultures, 80% of these patients displayed an increased C-reactive protein level, indicative of an infection and vegetations in 79% of cases (113). The first reported localized T. whipplei endocarditis case was diagnosed by broad-range bacterial PCR analysis (153).…”

“…Cardiovascular involvement. Endocarditis can also occur in the classic form of Whipple's disease and is usually present only in the late phase (113). This manifestation is often preceded by arthralgia or arthritis without gastrointestinal signs (132).…”

“…It is important that clinicians are aware of this, because up to 50% of the patients with classic Whipple's disease were initially misdiagnosed and given antirheumatic agents like disease-modifying antirheumatic drugs (DMARDs), anti-necrosis factor alpha, glucocorticoids, or drugs that can enhance the spread of the infection and thus may have fatal consequences (102,111). In retrospect, physicians should consider occult T. whipplei infections in patients treated for unrelated bacterial infections showing a surprising decrease in their previously unexplained arthralgia (112,113).…”

“…The first reported localized T. whipplei endocarditis case was diagnosed by broad-range bacterial PCR analysis (153). As there are no evident diagnostic criteria, clinicians should be especially alert for a potential T. whipplei endocarditis when dealing with white men who are around 50 years of age with cardiac symptoms like heart failure, large vegetations, and destruction of the heart valve, acute ischemic stroke, and embolic events (103,113,154). Of all patients with blood culture-negative endocarditis, PCR revealed the presence of a T. whipplei infection in 1.9 to 6.3% of patients (103,113,155).…”