Whipple's disease is a chronic multiorgan infection caused by Tropheryma whipplei, a weakly staining gram-positive bacillus. Humans are the only known host. Seroprevalence studies indicate that ∼50% of people in Western Europe and ∼75% of those in rural Senegal have been exposed to T. whipplei, and the disease prevalence is estimated at 1-3 cases per 1 million population. The route of transmission is unclear but probably involves fecal-oral spread and possibly involves droplet and/or airborne transmission.
Exposure to T. whipplei typically results in asymptomatic carriage but can lead to acute disease or chronic infection (Whipple's disease).
- Acute infection: Acquisition of T. whipplei can result in fever, acute gastroenteritis, and/or pneumonia.
- Chronic infection: “Classic” Whipple's disease typically begins with oligo- or polyarthralgias/seronegative arthritis. GI signs and symptoms (diarrhea, weight loss, abdominal pain) due to involvement of the duodenum and/or jejunum begin, on average, 6-8 years later. Other organ systems (e.g., the CNS and the cardiac, pulmonary, and lymphatic systems) are variably involved.
The key to diagnosis is considering T. whipplei infection. PCR-based testing of tissue specimens rather than body fluids generally has a higher diagnostic yield. Histologic examination of intestinal biopsy samples remains an important diagnostic procedure, although it is less sensitive than PCR. Serology is of little value in diagnosing active infection.
| TREATMENT | ||
Whipple's DiseaseAlthough the optimal regimen and duration are not known (and likely depend on the site of infection), ceftriaxone (2 g IV q24h) or meropenem (1 g IV q8h) for 2 weeks followed by TMP-SMX (160/800 mg PO bid) for 3-12 months appears to be efficacious. For CNS or cardiac infection, ceftriaxone (2 g IV q12h) or meropenem (2 g IV q8h) for 2-4 weeks followed by oral doxycycline or minocycline plus hydroxychloroquine or chloroquine for ≥1 year seems prudent. |