Whipple’s disease: a review

Abstract

SUMMARY
Whipples 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.
Its 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 Whipples 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
Whipples 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. Whipples 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.
Key Words: Whipples disease, Tropheryma whipplei,
treatment