Abstract

Whipple disease is a rare chronic infection due to Trophyrema whipplei, potentially lethal in the absence of appropriate anti-infective treatment. The clinical presentation is either of classical Whipple disease or of localized infection. Symptoms of classic Whipple disease include weight loss, diarrhea and arthralgia. Arthropathy is usually migratory, seronegative and non-destructive joint disease affecting preferentially large peripheral joints.Polymorphous and non-specific symptoms and signs, combined with its low incidence and the failure to grow on standard culture media make Whipple disease a hard-to-diagnose infection, with a diagnosis delay of several years after symptoms onset in most cases. The diagnosis of classical Whipple's disease is based on PCR using sample fluids (stool, saliva, urine and blood) and has to be confirmed by duodenal biopsies.To date, there are no guidelines available for the treatment of T. whipplei infections. Current anti-infective treatment is based on third generation cephalosporins on initial course, and then be followed by oral doxycyclin (or sulfamethoxazole-trimethoprim) for at least twelve months. Relapses and immune reconstitution inflammatory syndrome are the main complications.

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