[Whipple's disease: molecular biology--clinical aspects--therapy]

Whipple's disease is a rare systemic bacterial disease, affecting mostly middle-aged white men. Its clinical presentation is very variable. Arthralgias, fever of unknown origin or central nervous system symptoms may precede the more typical gastrointestinal manifestations with weight loss and chronic diarrhea. The diagnosis is based on the histopathologic demonstration of PAS-positive macrophages in duodenal or jejunal biopsies. In addition, it has become possible to detect the causative actinobacterium Tropheryma whippelii by amplification of a Whipple-specific PCR product. Therapeutic options are a sequential therapy with penicillin-streptomycin, followed by trimethoprim-sulfamethoxazole (TMP-SMX) or a monotherapy with TMP-SMX. These therapeutic modalities usually eradicate the infection and result in a rapid and sustained improvement of the Whipple-specific signs and symptoms. In view of the frequently uncharacteristic initial clinical presentation (arthralgias, fever, CNS symptoms), it is therefore of utmost importance to consider Whipple's disease in the differential diagnosis of the clinical signs and symptoms.