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Diagnosis and treatment of Whipple's disease
1Klinik für Innere Medizin, Krankenhaus Speyerhof, Heidelberg, Germany.
Abstract:
Whipple's disease is a rare systemic infectious disease. To date, it has neither been possible to culture the bacillus Tropheryma whippelii, nor to infect other individuals with the pathogen. Today the diagnosis is confirmed by means of polymerase chain reaction (PCR) technology. Typically, the material for the PCR analysis comes from the duodenum. The diagnosis can also be established in this way on the basis of other tissue, or the cerebrospinal fluid. Treatment should only be carried out with antibiotics which cross into the cerebrospinal fluid, since there can also be an unrecognised involvement of the CNS. At present, the favoured method of treatment is the daily parenteral administration of 1.2 million units of benzylpenicillin (penicillin G) and streptomycin 1 g for a period of 2 weeks. This is followed by treatment with cotrimoxazole (trimethoprim 160 mg and sulfamethoxazole 800 mg) twice daily for 1 to 2 years. The treatment should begin and end with a PCR analysis of cerebrospinal fluid, in order to definitively diagnose infection of the CNS with Whipple's disease and to document the disappearance of the bacillus from the CNS.
Insights
Whipple's disease, a rare infection by Tropheryma whippelii, is diagnosed using PCR, often on duodenal or cerebrospinal fluid. Treatment involves specific antibiotics targeting CNS involvement, with PCR monitoring disease clearance.
Area of Science:
- Infectious Diseases
- Microbiology
- Neurology
Background:
- Whipple's disease is a rare systemic bacterial infection caused by Tropheryma whippelii.
- The bacterium has proven difficult to culture and transmit experimentally.
- Diagnosis relies heavily on molecular methods due to culture limitations.
Purpose of the Study:
- To outline current diagnostic and treatment strategies for Whipple's disease.
- To emphasize the importance of considering central nervous system (CNS) involvement.
- To detail recommended antibiotic regimens and monitoring protocols.
Main Methods:
- Diagnosis is confirmed via polymerase chain reaction (PCR) on duodenal tissue, other tissues, or cerebrospinal fluid (CSF).
- Treatment requires antibiotics that penetrate the CNS, such as benzylpenicillin (penicillin G) and streptomycin, followed by cotrimoxazole.
- Treatment duration is lengthy, ranging from 1 to 2 years, with initial and final PCR analysis of CSF.
Main Results:
- PCR is the current gold standard for diagnosing Whipple's disease.
- Effective treatment necessitates antibiotics with CNS penetration to address potential neurological involvement.
- A combination therapy of parenteral benzylpenicillin/streptomycin followed by oral cotrimoxazole is the preferred regimen.
Conclusions:
- Early and accurate diagnosis of Whipple's disease is crucial, particularly for CNS involvement.
- Long-term antibiotic therapy is essential for successful treatment and eradication of Tropheryma whippelii.
- Monitoring treatment efficacy with CSF PCR is vital to confirm disease resolution.