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[Whipple disease: a single or multiple origin?]
M Cerf1, C Marche, J M Ciribilli
1CHU Bichat-Claude Bernard, Université Paris VII.
Abstract:
After having been considered as an essentially digestive disease, Whipple's disease has appeared more and more to be a multivisceral disease with two main characteristics: on one hand Whipple's disease yields a diffuse infiltration of tissues by abnormal macrophages without any other inflammatory reaction; on the other hand, aspects of microbial invasion by intra or extracellular unique rod-shaped Gram+bacteria are found. This unusual pathological complex has alternatively been considered as suggestive of an immunological defect or as a very unusual type of bacterial infection. Though recent studies support the hypothesis of a primary microbial infection due to a hitherto undescribed bacterium (Tropheryma whippelii) or more or less related bacteria belonging to the actinomycetes family, they do not totally exclude a primary or acquired impairment of antigen processing by macrophages. Speculations about this fascinating pathophysiological model and about its optimal therapeutic modalities are not likely to reach a conclusion in the near future.
Insights
Whipple's disease is a rare multivisceral condition characterized by abnormal macrophages and bacterial invasion. Current research suggests a primary infection by Tropheryma whippelii, though immune system defects are also considered.
Area of Science:
- Gastroenterology
- Infectious Diseases
- Immunology
Context:
- Whipple's disease was historically viewed as a digestive disorder.
- Emerging evidence highlights its systemic nature, affecting multiple organs.
- Characterized by abnormal macrophage infiltration and bacterial presence.
Purpose:
- To explore the complex pathophysiology of Whipple's disease.
- To differentiate between an infectious etiology and an immunological defect.
- To discuss potential therapeutic strategies.
Summary:
- Whipple's disease presents with diffuse tissue infiltration by macrophages and invasion by unique Gram-positive bacteria.
- The condition is increasingly recognized as multivisceral, not solely digestive.
- Current hypotheses favor a primary infection by Tropheryma whippelii or related actinomycetes, but impaired macrophage function remains a possibility.
Impact:
- Reframes the understanding of Whipple's disease from a digestive to a multivisceral condition.
- Highlights the role of Tropheryma whippelii in the disease's pathogenesis.
- Suggests ongoing research is needed to fully elucidate the pathophysiology and optimize treatment for Whipple's disease.