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Whipple's disease - a rare and challenging complication in a patient with Crohn's disease
Kerem Bulut1, Antoaneta Markova2, Ali E Canbay3
1Clinic for Internal Medicine and Gastroenterology, St. Clemens-Hospital Geldern, Germany.
Introduction:
Whipple's disease (WD) is a rare and infectious condition leading to multi-organ impairment caused by Tropheryma whipplei (TW), a ubiquitously occurring bacterium. TW can be detected in tissues by histological detection of PAS ("periodic acid-ship reaction")-positive macrophages and by polymerase-chain-reaction (PCR). Clinically, WD is often characterized by diarrhea, abdominal pain, and weight loss. These symptoms are also typical for a flare in Crohn's disease (CD) and, therefore, can lead to fatal misdiagnosis and wrong treatment by using biologics (e.g., anti-TNF-α).
Case Report:
We here report a young male patient with pre-existing CD. The patient's symptoms were misinterpreted as a flare of CD and illustrate the multifaceted nature of WD. After intensifying immunosuppressive therapy, the patient developed therapy-refractory diarrhea with several opportunistic infections with a final, fatal outcome.
Conclusion:
Patients with inflammatory bowel disease (IBD) are not only at risk from infectious complications known with clostridium difficile or cytomegalovirus (CMV); infection with WD should also be ruled out by endoscopy and biopsy before the escalation of the immunosuppressive regime.
Insights
Whipple's disease (WD), caused by Tropheryma whipplei, mimics Crohn's disease symptoms. Early diagnosis via endoscopy and biopsy is crucial to prevent fatal misdiagnosis and inappropriate immunosuppression in IBD patients.
Area of Science:
- Gastroenterology
- Infectious Diseases
- Microbiology
Background:
- Whipple's disease (WD) is a rare infectious condition caused by Tropheryma whipplei (TW), leading to multi-organ impairment.
- TW is detectable via PAS-positive macrophages and PCR in tissues.
- WD symptoms like diarrhea, abdominal pain, and weight loss overlap with Crohn's disease (CD) flares, risking misdiagnosis.
Observation:
- A young male patient with pre-existing CD presented with symptoms mimicking a CD flare.
- Intensified immunosuppressive therapy for presumed CD led to refractory diarrhea and opportunistic infections.
- The patient experienced a fatal outcome due to misdiagnosis and delayed/inappropriate treatment.
Findings:
- The case highlights the diagnostic challenge posed by WD in patients with inflammatory bowel disease (IBD).
- Misinterpreting WD as a CD flare can lead to escalating immunosuppression, worsening the infection.
- Timely and accurate diagnosis of WD is critical, especially before increasing immunosuppressive therapy.
Implications:
- Patients with IBD are susceptible to various infections, including WD, beyond C. difficile and CMV.
- Endoscopy with biopsy for WD detection is recommended before escalating immunosuppressive treatment in IBD patients.
- Ruling out WD is essential to prevent fatal outcomes in IBD patients presenting with overlapping symptoms.
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