Whipple’S Disease

(Whipple’s disease; Intestinal lipodystrophy)

The Whipple’s disease is a serious disease, which is caused by the bacterium Tropheryma whippelii. Leading symptoms include arthritis, weight loss, abdominal pain and diarrhea. The diagnosis is made with a small bowel biopsy. Initial treatment consists of the administration of ceftriaxone or penicillin, followed by trimethoprim / sulfamethoxazole for at least 1 year.

When Whipple’s disease is a malabsorption syndrome. It mainly affects white men aged between 30 and 60 years. Although he can manifest itself in many parts of the body (eg. As heart, lung, brain, serous cavities, joints, eyes, GIT), the intestinal mucosa is actually getting infected. These patients may have minor defects in cell-mediated immunity that predispose them to infection with T. whippelii. Approximately 30% of patients carry the HLA-B27.

The Whipple’s disease is a serious disease, which is caused by the bacterium Tropheryma whippelii. Leading symptoms include arthritis, weight loss, abdominal pain and diarrhea. The diagnosis is made with a small bowel biopsy. Initial treatment consists of the administration of ceftriaxone or penicillin, followed by trimethoprim / sulfamethoxazole for at least 1 year. When Whipple’s disease is a malabsorption syndrome. It mainly affects white men aged between 30 and 60 years. Although he can manifest itself in many parts of the body (eg. As heart, lung, brain, serous cavities, joints, eyes, GIT), the intestinal mucosa is actually getting infected. These patients may have minor defects in cell-mediated immunity that predispose them to infection with T. whippelii. Approximately 30% of patients carry the HLA-B27. Symptoms and signs The clinical manifestation is variable and depends on the affected organ system. The four main symptoms of Whipple’s disease are: arthralgia diarrhea abdominal pain weight loss As a rule, the first symptoms of arthritis and fever. Intestinal symptoms (eg., Aqueous diarrhea, steatorrhea, abdominal pain, anorexia, weight loss) are manifested mostly later, sometimes years after the first symptoms. It may be a visible or occult intestinal hemorrhage occur. Severe malabsorption may be in patients who are diagnosed with advanced disease. Other findings include increased skin pigmentation, anemia, lymphadenopathy, chronic cough, serositis, peripheral edema and CNS symptoms. Diagnostic endoscopy with small bowel biopsy One can overlook the diagnosis of Whipple’s disease in patients without prominent gastrointestinal symptoms. To a Whipple middle age should be considered among white men who have arthritis and abdominal pain, diarrhea, weight loss or other symptoms of malabsorption. These patients receive an endoscopy of the upper gastrointestinal tract with removal of a small bowel biopsy. The intestinal changes are specific and diagnostic. The most serious and typical of Whipple’s disease changes are made in the proximal small intestine. In light microscopy can see PAS-positive macrophages that distort the villous architecture. Gram-positive, non-acid-fast bacteria (T. whippelii) can be detected in the lamina propria and in the macrophages. If T. whipplei is not visible, but Whipple’s disease is still clinically suspected, PCR assays and immunohistochemistry should be performed. The Whipple’s disease should be differentiated from the intestinal infection with Mycobacterium avium-intracellulare (MAI) having similar histological findings. However, in May are acid-resistant. Antibiotics later relapse possible Untreated cases progressive and fatal. A number of antibiotics affects curative (z. B. trimethoprim / sulfamethoxazole, chloramphenicol, ampicillin, penicillin, cephalosporins). Treatment of Whipple’s disease is with ceftriaxone (2 g iv daily) or penicillin G (1.5-6 million units iv every 6 h) initiated. In this regime a long-term administration of trimethoprim / sulfamethoxazole follows (160/800 mg po 2 times a day for 1 year) or a combination of doxycycline (100 mg orally two times a day for 1 year) and hydroxychloroquine (200 mg po 3- times a day for 1 year). Patients who are allergic to sulfur must receive oral penicillin or ampicillin. Clinical improvement occurs very quickly, fever and joint pain disappear within a few days. The intestinal symptoms usually resolve within 1-4 weeks. To confirm the therapeutic response, a PCR test in the stool, saliva or other tissue can be performed. However, other experts recommend a repeat biopsy with microscopy after 1 year to document bacteria (not only macrophages, which may persist for years after successful treatment) in conjunction with a PCR test. Relapses are common and can occur even years later. If there is a suspicion of a relapse, small intestine biopsies are obtained or PCR assays performed (independently of the affected organ system) to detect possibly free bacteria. Summary Infection with the bacteria T. whippelii affects many organs, including the GIT. The involvement of the intestinal mucosa causes malabsorption. A suspicion of Whipple’s disease is among white middle-aged men who have arthritis and abdominal pain, diarrhea, weight loss or other symptoms of malabsorption. What is needed is an endoscopic small bowel biopsy. A long-term treatment with antibiotics is necessary, relapses are common.

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