Whipple disease

Overview
Whipple's disease is a rare, systemic infectious disease caused by the bacterium Tropheryma whipplei. First described by George Hoyt Whipple in 1907 and commonly considered a gastrointestinal disorder, Whipple's disease primarily causes malabsorption but may affect any part of the body including the heart, lungs, brain, joints, and eyes. Weight loss, incomplete breakdown of carbohydrates or fats, and malfunctions of the immune system are common presenting symptoms. When recognized and treated, Whipple's disease can usually be cured; untreated the disease may be fatal.

Symptoms

 * arthritis
 * malabsorption
 * intestinal lipodystrophy (accumulation of fatty deposits in lymph nodes of the intestine)
 * lymphadenopathy
 * abdominal pain
 * diarrhea
 * fever
 * neurological symptoms
 * cognitive changes
 * nystagmus

Diagnosis
Symptoms of Whipple's disease include joint problems, reduced vision, and intestinal inflammation (including diarrhea). Diagnosis is made by intestinal biopsy, which shows as PAS-positive macrophage inclusions.

At times, small bowel X-rays may show some thickened folds.

Treatment
Treatment is with penicillin, ampicillin, tetracycline or co-trimoxazole for 1 -2 years. Any treatment lasting less than a year has an approximate relapse rate of 40%. In the January 4, 2007 issue of the New England Journal of Medicine, Fenollar et al suggest the use of doxycycline with hydroxychloroquine for 12 to 18 months. Sulfonamides (Sulfadiazine or Sulfamethoxazole) should be added for treatment of neurological symptoms.

Acknowledgements
The content on this page was first contributed by: C. Michael Gibson, M.S., M.D.

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