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Whipple's disease: case report and review of the literature
Vincenzo De Francesco1, Fabrizio Corsi2, Antonio Pennella2
1Section of Gastroenterology, Department of Medical Sciences, Foggia, Italy.vdefrancesco@ospedaliriunitifoggia.it.
Abstract:
Whipple's disease (WD) is known as an infrequent, systemic, chronic infection caused by the actinomycete Tropherima whipplei (T. whipplei). The disease is frequently characterized by a long prodromal and protean extra-intestinal phase, which often causes misdiagnosis and inappropriate treatments. Herein, we describe the case a 62-year-old man with a histological diagnosis of WD established when oral steroid treatment was started due to rheumatic manifestations, triggering intestinal symptoms. Systematic review of the literature was performed to include studies where WD was eventually diagnosed on duodenal biopsies. Three patients' subgroups were identified according to the clinical presentation.
Insights
Whipple's disease (WD), a chronic infection by Tropherima whipplei, often presents with rheumatic symptoms before intestinal issues arise. Steroid treatment can unmask or worsen these gastrointestinal manifestations, highlighting diagnostic challenges.
Area of Science:
- Infectious Diseases
- Gastroenterology
- Rheumatology
Background:
- Whipple's disease (WD) is a rare systemic infection caused by Tropherima whipplei.
- It often features a prolonged prodromal phase and diverse extra-intestinal symptoms, leading to diagnostic delays and misdiagnosis.
- Rheumatic manifestations can precede overt gastrointestinal symptoms, complicating early identification.
Observation:
- A 62-year-old man received a histological diagnosis of WD after initiating oral steroid therapy for rheumatic symptoms.
- The steroid treatment precipitated gastrointestinal symptoms, leading to the WD diagnosis.
- A systematic literature review identified three patient subgroups based on clinical presentation.
Findings:
- The case illustrates a specific trigger for WD symptom manifestation.
- Literature review supports the heterogeneity of WD clinical presentations.
- Duodenal biopsies are crucial for diagnosing WD, even with initial non-gastrointestinal complaints.
Implications:
- Early recognition of WD's protean manifestations is critical for timely and accurate diagnosis.
- Understanding triggers like immunosuppression may aid in earlier WD detection.
- Further research into WD subgroups can refine diagnostic and treatment strategies.
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