Whipple's disease in a man of North African descent : case report and brief review of the literature

Acta Gastroenterol Belg. 2019 Jan-Mar;82(1):83-86.

Abstract

A 62-year-old man of North African descent presented with weight loss in the past year and diarrhea for three weeks. His medical history included erosive rheumatoid arthritis, treated with methotrexate and adalimumab. Histological examination of a duodenal biopsy showed foamy macrophages in the lamina propria, with PAS-positive cytoplasmatic inclusions. These findings are compatible with Whipple's disease, a rare chronic infectious disease caused by Tropheryma whipplei, an opportunistic bacterium. It is typically seen in middle-aged Caucasian men and the immunocompromised host. The classical presentation of Whipple's disease consists of intermittent migratory arthralgia, followed by intestinal symptoms which typically occur six to seven years later. The clinical image can be very variable, and this complicates the diagnostic process. PAS-staining and PCR are the diagnostic cornerstones. In our case, treatment consisted of a prolonged cure of antibiotics: intravenous ceftriaxone for two weeks, followed by an oral maintenance therapy of doxycycline and hydroxychloroquine for at least one year. A therapeutic dilemma arose as continued anti-TNF blockade was necessary to maintain remission of the rheumatoid arthritis. Lifelong follow-up is necessary because relapse is possible.

Keywords: Tropheryma whipplei; Whipple’s disease; clinical features; periodic acid-Schiff.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anti-Bacterial Agents / therapeutic use*
  • Ceftriaxone / therapeutic use
  • Doxycycline / therapeutic use
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Male
  • Middle Aged
  • Treatment Outcome
  • Tropheryma / isolation & purification*
  • Tumor Necrosis Factor-alpha
  • Whipple Disease / diagnosis*
  • Whipple Disease / drug therapy*

Substances

  • Anti-Bacterial Agents
  • Tumor Necrosis Factor-alpha
  • Hydroxychloroquine
  • Ceftriaxone
  • Doxycycline