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Whipple's disease
Last reviewed: 23.04.2024
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Whipple's disease is a rare systemic disease caused by the bacterium Tropheryma whippelii. The main symptoms of Whipple's disease are arthritis, weight loss and diarrhea. Diagnosis is established by a biopsy of the small intestine. Treatment of Whipple's disease consists in taking at least 1 year of trimethoprim-sulfamethoxazole.
Whipple's disease mainly affects people with white skin color at the age of 30-60 years. Although this disease affects many organs (eg, heart, lung, brain, serous cavities, joints, eyes, GI tract), almost always the mucosa of the small intestine is involved in the process. Patients may have weakly expressed cellular immunity defects that predispose to infection of T. Whippelii. Approximately 30% of patients have HLAB27.
What causes Whipple's disease
In 1992, the bacterial nature of Whipple's disease was established (Relman, Schmidt, MacDermott, 1992). As an infectious agent identified Gram-positive actinomycetes Tropheryna whippelii. These small Gram - positive bacteria are found in the active phase of the disease in large quantities in the mucosa of the small intestine and other organs and disappear after intensive antibacterial treatment. Predisposing to the development of the disease factor is a violation of the immune system of different genesis.
Symptoms of Whipple's Disease
The clinical symptoms of Whipple's disease vary depending on the affected organs and systems. Usually the first signs are arthritis and fever. Intestinal symptoms of Whipple's disease (eg, watery diarrhea, steatorrhea, abdominal pain, anorexia, weight loss) usually manifest later, sometimes several years after the initial complaints. There may be profuse or latent intestinal bleeding. Severe malabsorption can appear and be diagnosed in patients in the late stages of the clinical course. Other results of the examination include increased skin pigmentation, anemia, lymphadenopathy, chronic cough, polyserositis, peripheral edema and signs of CNS damage.
Diagnosis of Whipple's disease
The diagnosis can be unverified in patients without visible symptoms from the digestive tract. Whipple's disease should be suspected in middle-aged Caucasian people with arthritis, abdominal pain, diarrhea, weight loss, or other signs of malabsorption. Such patients need to perform endoscopy of the upper digestive tract with a biopsy of the small intestine; lesions of the intestine are specific and allow diagnosis. The most significant and persistent changes affect the proximal parts of the small intestine. Light microscopy allows you to visualize PAS-positive macrophages that deform the villain architectonics. Gram-positive, negative when stained with acid dye bacilli (T. Whippelii) are determined in their own plate and in macrophages. Electron microscopy is recommended.
Whipple's disease should be differentiated with an intestinal infection caused by Mycobacterium avium-intracellulare (MAI), which has similar histological signs. However, MAI stains positively when treated with an acidic dye. A polymerase chain reaction may be useful to confirm the diagnosis.
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Treatment of Whipple's Disease
Without treatment Whipple's disease progresses and can be fatal. Various antibiotics are effective (eg, tetracycline, trimethoprim-sulfamethoxazole, chloramphenicol, ampicillin, penicillin, cephalosporins). One of the recommended regimens is: ceftriaxone (2 g IV daily) or procaine (1.2 million units IM once a day), or penicillin G (1.5-6 million units IV every 6 hours) together with streptomycin (1.0 g IM once a day for 10-14 days) and trimethoprim-sulfamethoxazole (160/800 mg orally 2 times a day for 1 year). Patients with an allergy to sulfonamides, these drugs can be replaced by oral administration of penicillin VK or ampicillin. Clinical improvement occurs quickly, fever and joint pain are resolved after a few days. Intestinal symptoms of Whipple's disease usually disappear within 1-4 weeks.
Some authors do not recommend performing repeated biopsies of the small intestine, arguing that macrophages can persist for several years after treatment. However, other authors recommend a repeat biopsy after the first year of treatment. In the latter case, electron microscopy is necessary to document the presence of bacilli (not just macrophages). Relapses of the disease are possible even after years. If relapse is suspected, a small bowel biopsy (regardless of organ or system damage) is necessary to verify the presence of free bacilli.