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Clinical Features of Whipple Disease: The patients develop migratory polyarthritis (affecting large joints; rheumatoid factor negative), abdominal pain, diarrhea, weakness and gradual weight loss. Steatorrhea develops in late stages of the disease.

CNS involvement can present with cognitive dysfunction, dementia, ophthalmoplegia, myoclonus, hypothalamic damage with dysautonomia, oculomasticatory or oculo-facial-skeletal myorhythmia, ataxia, seizures, and psychiatric changes.

Cardiac manifestations include culture-negative endocarditis. There is marked enlargement of mesenteric and periaortic lymph nodes. Generalized skin hyperpigmentation may also be present.

The image of Whipple disease involving a lymph node shows ill-defined granulomas, cystic spaces, and aggregates of foamy histiocytes. Image courtesy of: ARP Press; used with permission

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