Chapter 11 : Whipple’s Disease

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Whipple’s disease (WD) is a rare multisystemic chronic infection caused by the rod-shaped bacterium . Patients usually present with symptoms of weight loss, arthralgia, diarrhea, and abdominal pain. Involvement of the heart, lung, and central nervous system (CNS) is also common. The diagnosis is established by small-bowel biopsy in which the samples show inclusions in macrophages of the lamina propria staining with periodic acid-Schiff (PAS). The inclusions present remnants of the causative organism . Phylogenetic analysis of the 16S rRNA gene sequence established that this bacterium is an actinomycete. In patients already infected with , immunosuppression accelerates the course of the infection and especially triggers the occurrence of intestinal manifestations. Neurologic manifestations occur in three situations: neurologic involvement in classic WD, isolated neurologic symptoms due to infection without histologic evidence of intestinal involvement, and neurologic relapse of previously treated WD. The pathogenesis of cerebral infarction is not well established, but cerebral vasculitis, arterial fibrosis, thrombosis, and thickening associated with the inflammation of adjacent brain parenchyma and leptomeninges, caused by the hematogenous spread of to the brain, may all be important triggers. Immunohistochemical staining for antibodies against can detect the organism in various tissues, body fluids, and blood monocytes. Therapeutic recommendations are empirically based or based on in vitro susceptibility tests. Due to the rarity of the disorder, cooperative studies and clinical trials are necessary to clarify the unanswered questions and to establish evidence-based therapeutic recommendations.

Citation: Marth T, Deriban G. 2009. Whipple’s Disease, p 205-216. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch11
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Citation: Marth T, Deriban G. 2009. Whipple’s Disease, p 205-216. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch11
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Table 1.

Percentage of individuals with Whipple’s disease showing specific clinical features

Citation: Marth T, Deriban G. 2009. Whipple’s Disease, p 205-216. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch11
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Table 2.

Long-term consequences of infection with

Citation: Marth T, Deriban G. 2009. Whipple’s Disease, p 205-216. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch11

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