Whipple's disease history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
- Patients with Whipple's disease have varied presentation.
- Most common symptoms of Classical Whipple's disease include joint pain weight loss diarrhea and arthropathy. Various other organ systems can be involved in Whipple's disease including central nervous system, cardiac system, renal system, skeletal muscles and pulmonary system.
History and Symptoms
Patients with Classical Whipple's Disease often present with weight loss with diarrhea. Diarrhea is the most common complain in the patients with Whipple's disease and it is associated with Steatorrhea. Other gastrointestinal symptoms may be often seen like abdominal pain, occult mucosal bleeding, ascites, hepatospleenomegaly and occasionally hepatitis.
Joint involvement is another predominant complain in patients with Whipple's disease. It is second most commonly involved system after the gastrointestinal system. Patients may present with intermittent migratory arthralgias and/or arthritis often affecting peripheral joints like knees elbows wrists etc. Multiple joint involvement is common. Most attacks are acute, rarely chronic arthritis may be destructive.
Skeletal muscle involvement in Whipples disease is seen as myalgias and muscle cramps
Neurologic involvement is seen in 6 to 63% of patients and patients can present with wide variety of symptoms. Most often patients present with congnitive changes. Som patients may present with psychiatric symptoms like depression and personality changes. About fifty percent patients may have supranuclear opthalmoplegia at presentation and twenty five percent of the patients may present with myoclonus. On rare occasion patients may present with hypothalmic involvement seen as polydipsia, hyperphagia, change in libido and sleep wake cycle.
The pathognomic abnormalities in Whipple's are eye movement abnormalities.
Central nervous system involvement in Whipple's disease may present as asymptomatic involvement with detection of T. whipplei DNA in CSF or it may present as ocular involvement like anterior or posterior uveitis. CNS involvement usually has poor prognosis.
Some patients may present with cardiac involvement which has varied presentation ranging from Pericarditis, Myocarditis, heart failure or Sudden Cardiac death. Endocarditis associated with negative blood cultures. Endocarditis is often preceeded by arthritis and arthralgias.
Pulmonary involvement in classic Whipple’s disease may manifest as pleural effusion, pulmonary infiltration, or granulomatous mediastinal
adenopathy. Often abdominal lymph nodes may be involved.
Classical cutaneous finding reported in Whipple's disease is Melanoderma. this is less frequently reported these days as Whipple's disease is increasingly recognized early.
Late in course renal involvement may be seen rarely.
References
http://www.nejm.org/doi/full/10.1056/NEJMra062477