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Whipple's disease Microchapters |
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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
Non specific symptoms
Gastrointestinal system
- Diarrhea: The most common complaint
- Steatorrhea
- Abdominal pain
- Occult mucosal bleeding
- Ascites
- Hepatosplenomegaly
- Hepatitis
Articular system
- Intermittent migratory arthralgias: The second most common complaint
- arthritis: affecting peripheral joints like knees elbows wrists
- Multiple joint involvement
Less Common Symptoms
- 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. [1] [2]
- 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. [2]
- Skeletal muscle involvement in Whipples disease is seen as myalgias and muscle cramps [1]
- 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 preceded by arthritis and arthralgias .[1] [2]
- 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. [2]
- Hematological finding in Whipple's disease is anemia which may be due to malabsorption or mucointestinal bleeding.[2]
- 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. [1]
- Late in course renal involvement may be seen rarely. [1]
- Most attacks are acute, rarely chronic arthritis may be destructive. [1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Fenollar F, Puéchal X, Raoult D (2007). "Whipple's disease". N Engl J Med. 356 (1): 55–66. doi:10.1056/NEJMra062477. PMID 17202456.
- ↑ 2.0 2.1 2.2 2.3 2.4 Dutly F, Altwegg M (2001). "Whipple's disease and "Tropheryma whippelii"". Clin Microbiol Rev. 14 (3): 561–83. doi:10.1128/CMR.14.3.561-583.2001. PMC 88990. PMID 11432814.