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: Bhagyashree Deshmankar M.B.B.S.[2]
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
Common Symptoms
- Gastrointestinal system : 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. [1]
- Articular system : 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.
- Central nervous system : CNS involvement in Whipple's disease most often presents as asymptomatic involvement with detection of T. whipplei DNA in CSF. In patients with symptomatic disease there might be focal CNS involvement which determine the varied clinical symptoms like dementia, cerebral ataxia,hemiparesis, personality changes, opthalmoplegia, nystagmus etc. 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 Oculomasticatory myorhythmia (OMM) and oculofacial-skeletal myorhythmia (OSFM). CNS involvement usually has poor prognosis.
Less Common Symptoms
- Ocular involvement in Whipple's disease is rare and can involve uveitis, retinitis, vitritis, retrobulbar neuritis, papilledema. Ocular involvement is often seen in patients with CNS disease.
- Skeletal muscle involvement in Whipples disease is seen as myalgias and muscle cramps
- Some patients may present with cardiac involvement which has varied presentation ranging from Pericarditis, Myocarditis, heart failure or Sudden Cardiac death. Endocarditis due to Whipple's disease is associated with negative blood cultures. Endocarditis is often preceeded by arthritis and arthralgias.
- Pulmonary involvement in classic Whipple’s disease may manifest as pleuritic chest pain, non productive cough and dyspnea. Chest x-ray often shows pleural effusion, pulmonary infiltration, or granulomatous mediastinal adenopathy. Often abdominal lymph nodes may be involved.
- Hematological finding in Whipple's disease is Anemia which may be due to malabsorption or mucointestinal bleeding.
- 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
http://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.320.992&rep=rep1&type=pdf
- ↑ La Scola, B; Altwegg, M; Mallet, M N; Fournier, P E; Fenollar, F; Raoult, D (2001). "Description of Tropheryma whipplei gen. nov., sp. nov., the Whipple's disease bacillus". International Journal of Systematic and Evolutionary Microbiology. 51 (4): 1471–1479. doi:10.1099/00207713-51-4-1471. ISSN 1466-5026.