[Whipple disease--a rare systemic disease].

Whipple's disease is a rare, without antibiotic treatment deadly systemic infectious disease caused by the ubiquitary Gram positive bacterium Tropheryma whipplei. The agent can be demonstrated by light and electron microscopy and by PCR in tissues and body fluids affected. Men are affected more often than women. Most patients lose weight, have diarrhea, abdominal pain and arthralgias. In 10-40% of cases involvement of the CNS, often asymptomatic, is observed. PAS-positive macrophages in the lamina propria of duodenal biopsies are typical but not pathognomonic of Whipple's disease. Nowadays, the infectious agent should also be demonstrated by PCR. Treatment, mostly as a sequential therapy, is with antibiotics that cross the blood-brain barrier and should last for at least 1 year in order to avoid relapses. Before ending therapy the absence of Tropheryma whipplei in duodenal tissue and cerebrospinal fluid must be confirmed.