[The rich diversity of Whipple's disease]

M Sluszniak1, I H Tarner2,3, A Thiele4

  • 1Klinik für Rheumatologie, Immunologie und Osteologie, Krankenhaus St. Josef Wuppertal, Bergstr. 6-12, 42105, Wuppertal, Deutschland. Marta.Sluszniak@cellitinnen.de.

Insights

Whipple's disease (WD), caused by Tropheryma whipplei, presents diverse symptoms beyond gastrointestinal issues, often mimicking other conditions. Early detection via histology or PCR in affected organs is crucial for timely antibiotic treatment.

Area of Science:

  • Infectious Diseases
  • Rheumatology
  • Gastroenterology

Background:

  • Whipple's disease (WD) is a rare, chronic, multiorgan bacterial infection caused by Tropheryma whipplei.
  • Clinical presentation is highly polymorphic, frequently involving gastrointestinal symptoms but also presenting with arthritis, cardiac, or neurological manifestations.
  • Misdiagnosis is common, particularly as seronegative rheumatoid arthritis, leading to inappropriate immunosuppressive therapy.

Purpose of the Study:

  • To illustrate the diverse clinical presentations of Whipple's disease.
  • To highlight the importance of considering WD even without gastrointestinal symptoms.
  • To emphasize the need for direct detection of Tropheryma whipplei in affected organs.

Main Methods:

  • Histological examination using periodic acid-Schiff (PAS) staining to identify PAS-positive macrophages.
  • Polymerase chain reaction (PCR) for Tropheryma whipplei detection.
  • Case report analysis of three patients with distinct initial symptoms.

Main Results:

  • The three reported cases presented with leading symptoms mimicking seronegative rheumatoid arthritis, spondyloarthritis, and adult-onset Still's disease.
  • These cases underscore that Whipple's disease can manifest without typical gastrointestinal involvement.
  • Diagnostic challenges arise due to the varied clinical spectrum.

Conclusions:

  • Whipple's disease requires a high index of suspicion, especially when symptoms are atypical or mimic other rheumatological conditions.
  • Direct detection of Tropheryma whipplei in affected tissues is essential for accurate diagnosis.
  • Recommended treatment involves antibiotics like ceftriaxone followed by cotrimoxazole for several months.

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