Whipples disease: a review

Whipple’s disease was described in 1907 and given the name “intestinal lipodystrophy” until it was found that the agent responsible is a bacterium named Tropheryma whipplei. It’s a rare disease which occurs predominantly in males aged 30-60. The small intestinal mucosa is always affected with lesions that are specific to this disease. Replacement of most of cellular elements in the lamina propria by macrophages is characteristic of Whipple’s disease. It is a systemic disease that can affect every system, usually causing symptoms in the bowel, the joints, the central nervous and the cardiovascular systems. The diagnosis of Whipple’s disease is not easy and depends on a combination of clinical features, the characteristic histopathological findings, the presence of pathognomonic PAS positive macrophages and the PCR of the 16S ribosomal RNA of Tropheryma whipplei. Whipple’s disease is lethal if not treated, though it responds dramatically to antibiotic treatment. Patients should be closely monitored during and after treatment because relapses are not uncommon especially when CNS is involved.