From the Guidelines
Whipple's disease is a rare bacterial infection caused by Tropheryma whipplei that primarily affects the small intestine but can spread throughout the body, and the standard treatment involves antibiotics, typically starting with intravenous ceftriaxone or penicillin G, followed by oral trimethoprim-sulfamethoxazole for 1-2 years, as recommended by the Infectious Diseases Society of America 1. The disease can cause a range of symptoms, including:
- Diarrhea
- Weight loss
- Joint pain
- Abdominal pain
- Heart and brain involvement Diagnosis usually requires intestinal biopsy showing PAS-positive macrophages containing the bacteria, and positive PCR for Tropheryma whipplei 1. Without treatment, Whipple's disease is potentially fatal, but with appropriate antibiotic therapy, most patients recover completely, however, relapses can occur, especially if treatment is stopped too early, which is why the prolonged antibiotic course is necessary 1. Regular follow-up is essential to monitor for recurrence, particularly in the first few years after treatment completion. The disease is more common in middle-aged white men and likely involves genetic factors affecting immune response to the bacteria. It is essential to note that Whipple's disease can be mistaken for other conditions, such as seronegative enteropathies, and a thorough diagnosis and treatment plan should be established by a healthcare professional 1.
From the Research
Overview of Whipple's Disease
- Whipple's disease is a rare, chronic, and systemic infectious disorder caused by the bacterium Tropheryma whipplei 2, 3, 4.
- The disease can affect multiple organs and systems, including the gastrointestinal tract, joints, and central nervous system 2, 3, 4.
Clinical Features
- The clinical manifestations of Whipple's disease are variable and can include weight loss, chronic diarrhea, arthralgias, and low-grade fever 2, 3, 4.
- Gastrointestinal symptoms, such as abdominal pain and diarrhea, are common, while neurological symptoms, such as cognitive impairment and memory loss, can occur in advanced cases 2, 4.
Diagnosis
- The diagnosis of Whipple's disease is typically made by finding PAS-positive macrophages in the lamina propria at duodenal biopsy 2, 3, 4.
- PCR for Tropheryma whipplei can also be used to diagnose the disease, but false positives can occur 2, 4.
- Immunohistochemistry and DNA sequencing can also be used to confirm the diagnosis 4.
Treatment
- The treatment of Whipple's disease typically involves antibiotic therapy, with ceftriaxone and trimethoprim-sulfamethoxazole being commonly used 5, 3, 6.
- Oral doxycycline and hydroxychloroquine have also been shown to be effective in treating Whipple's disease 5, 6.
- The optimal duration of therapy is not established, but lifelong prophylaxis with doxycycline may be necessary to prevent reinfections or reactivations 6.