Whipple disease is a rare infectious multisystem disorder caused by the actinobacteria Tropheryma whipplei.
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Epidemiology
The incidence of Whipple disease is not truly known, one Swiss study estimated it at approximately 1 per 1.5 million per year 7.
The peak age for presentation is in the fifth decade of life. Caucasians are most often affected, and men are affected eight times more commonly than women 5. The disease is more common in farmers and those who work with soil and livestock 8.
Clinical presentation
Patients often present with a non-destructive migratory arthritis, weight loss, diarrhea and abdominal pain. Less commonly patients present with fever, lymphadenopathy or hepatosplenomegaly 5. The presence of arthritis may precede other symptoms by years 8.
Pathology
A suspected diagnosis of Whipple disease can be confirmed by showing periodic acid-Schiff-positive granular foamy macrophages from sampled tissue, such as the small bowel or a peripheral lymph node 5.
Location
The small bowel (intestinal lipodystrophy) is a classical location although the disease can affect a multitude of other organ systems with or without small bowel involvement. Other locations include 1,2:
skin
joints
central nervous system
mediastinum
Radiographic features
These largely depend on the organ system involved:
Treatment and prognosis
CNS involvement with Whipple disease carries poor prognosis and it is invariably fatal without treatment. Approximately half of the patients may show some symptomatic improvement during antibiotic treatment. ~17.5% (range 2-33%) patients may relapse. Thus, early diagnosis and treatment are paramount for survival 6.
History and etymology
First described by George Hoyt Whipple, American (US) pathologist (1878-1976) in 1907 4.
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