Successful treatment of Whipple disease diagnosed 36 years after symptom onset

Whipple disease is a rare infectious disorder with multiorgan manifestation s and a widely varied clinical presentation. Involvement of the small intes tine with resultant malabsorption is a classic finding, although extraintes tinal manifestations such as fever and arthralgias may precede gastrointest inal symptoms by many years. We describe a 63-year-old man in whom Whipple disease was diagnosed 22 years after his initial presentation (36 years aft er symptom onset) with lymphadenopathy, when a biopsy yielded nonnecrotizin g granulomas. His recent symptoms included persistent fatigue, weight loss, fever, and arthralgias. Endoscopic biopsy specimens from the distal duoden um showed features consistent with Whipple disease, and Tropheryma whippeli i DNA was detected in both the small bowel biopsy specimen and the blood sp ecimen by polymerase chain reaction and DNA probe hybridization. His sympto ms resolved with long-term co-trimoxazole therapy. We discuss the protean m anifestations of Whipple disease, the difficulties in clinical diagnosis, a nd the recent advances in the molecular diagnosis of this disorder.