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A rare and chronic infection occurring primarily in Caucasian males and caused by the microorganism Tropheryma whipplei, Whipple disease involves the small intestine, where it leads to malabsorption, but it also causes a systemic infection with extraintestinal signs and symptoms. It is currently thought that an abnormal host response may play a central role in the pathogenesis of the disease, as the monocyte/macrophage function appears impaired. The clinical manifestations vary widely: as in the majority of cases the small intestine is involved, diarrhea is a predominant symptom, often associated with various degrees of malabsorption and fatigue. Extraintestinal manifestations are less common but are well described. They include seronegative arthritis, fever or neurological symptoms. Whipple disease should be considered in the differential diagnosis of malabsorption as well as in patients with unexplained weight loss, arthritis, culture negative endocarditis and fever of unknown origin. Endoscopy with small intestinal mucosal biopsy is the diagnostic test of choice and is required for a definitive diagnosis. Treatment consists in a prolonged course of antibiotic therapy. Many antibiotic regimens effective against gram-positive organisms have been used successfully to treat Whipple disease. In patients who complete a course of effective antibiotic therapy, the prognosis is excellent. Extraintestinal symptoms often disappear within a few days and gastrointestinal symptoms frequently resolve within 1 month. Within a few months, most patients are asymptomatic. However, despite the initial response to antibiotic therapy, relapses are common: they can occur during treatment or even months to years after its completion. [ABSTRACT FROM AUTHOR]