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The biopsy is figure A, and the treatment is antibiotics
7%
6/86
The biopsy is figure B, and the treatment is antibiotics
51%
44/86
The biopsy is figure A, and the treatment is avoidance of gluten. The patient is at greater risk of lymphoma
21%
18/86
The biopsy is figure B, and the treatment is avoidance of gluten. The patient is at greater risk of lymphoma
9%
8/86
The biopsy is figure C, and the treatment is surgical resection
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Whipple's Disease is a systemic illness consisting of arthralgia, weight loss, fever, diarrhea, and abdominal pain. The diagnosis is made by detecting PAS-positive material in the lamina propria of the small intestine on biopsy and treatment is with antibiotics. Infection with the gram positive bacterium Tropheryma whipplei may be detected by PAS positive foamy macrophages in the intestinal lamina propria and mesenteric lymph nodes. While Whipple's disease primarily affects the gastrointestinal tract - resulting in a malabsorptive diarrhea - it may affect almost any organ. There are a number of symptoms including cardiac manifestations, arthralgias and neurologic symptoms (including the brain and eye). The classic clinical presentation will be an older man with weight loss, diarrhea, joint pain, and arthritis. Further, the disease is more common in farmers and those exposed to soil and animals. Treatment involves antibiotics: penicillin, ampicillin, tetracycline, doxycycline, hydroxychloroquine, or co-tirmoxazole for several years. If not treated, the disease may be fatal. Note that while celiac disease may also cause a malabsorptive diarrhea along with other systemic manifestations such as dermatologic findings and anemia, the constellation of these symptoms - especially with arthralgia and fever, in a farmer are more suggestive of Whipple's. Moreover, celiac disease increases the risk for lymphoma while Whipple's does not. Juckett and Trivedi discuss the evaluation of chronic diarrhea. It may be separated into three categories: watery, fatty (malabsorptive), and inflammatory. Malabsorptive diarrhea is characterized by excess gas, steatorrhea, and weight loss. Giardiasis and Celiac disease are the classic examples. Celiac disease also results in weight loss and iron deficiency anemia. Whipple's disease is a less common cause of malabsorptive diarrhea. Schwartzman and Schwartzman discuss Whipple's disease. Diagnosis is confirmed by tissue sampling with periodic acid-Schiff staining and or polymerase chain reaction. The clinical manifestations are most often in the gastrointestinal tract, musculoskeletal system, neurologic system, heart, and eyes but can affect any sight. Even after successful treatment with antibiotics, the disease may recur. Image A depicts the biopsy of a patient with Celiac sprue. Note the blunting of the villi and crypt hyperplasia. Image B depicts the biopsy of a patient with Whipple's disease. Note the PAS positive foamy macrophages. Image C depicts the biopsy of a patient with an adenomatous polyp. Incorrect Answers: Answers 1 and 3: These biopsies depicts Celiac sprue (for which the treatment is avoidance of gluten). Answer 4: This biopsy does depict Whipple's disease but the treatment is antibiotics, not avoidance of gluten. Answer 5: Surgical resection is the treatment for an adenomatous polyp but this is not the disease that describes the symptoms of the patient in this vignette.
3.7
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