Tropheryma whipplei

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Tropheryma whipplei /
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Background

Microbiology

  • Fastidious Gram-positive bacillus

Epidemiology

  • Ubiquitous environmental organism, but rarely causes disease
    • Can be found in saliva of one third of healthy people
  • Disease is more common in white European males

Pathophysiology

  • Lack of host immune response

Clinical Manifestations

Classic Whipple's Disease

  • Cardinal features include: arthralgias, followed by weight loss, diarrhea, and abdominal pain
  • Arthralgias are typically migratory in the larger peripheral joints, including knees, ankles, and wrists, but can have essentially any presentation
  • The diarrhea is intermittent, with colicky abdominal pain
    • Diarrhea can be watery or have steatorrhea
    • Occasional GI bleeding

Other Symptoms

Diagnosis

  • Samples should be taken from involved sites, with a strong preference for small bowel biopsy if there are GI symptoms, but also synovial or cerebrospinal fluid or heart valve
    • Up to 7 to 10 biopsies from small bowel, ideally
  • Diagnosis is based on:
    • Periodic acid-Schiff-positive bacilli in macrophages
    • PCR
    • Immunohistochemistry

Management

Prognosis

  • Clinical improvement takes 1 to 3 weeks of treatment
  • Neurologic sequelae may be permanent
  • Relapses after treatment, including of CNS disease, can happen in up to a third of patients