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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
- Asymptomatic involvement with detection of T. whipplei DNA in CSF: most common finding.
- Focal CNS involvement may present with clinical symptoms like
- dementia
- cerebral ataxia
- hemiparesis
- personality changes
- opthalmoplegia
- nystagmus
- supranuclear opthalmoplegia at presentation in about 50% patients
- myoclonus. at presentation in about 25%
- Hypothalmic involvement seen as polydipsia , hyperphagia , change in libido and sleep wake cycle: rarely
- :Eye movement abnormalities Oculomasticatory myorhythmia (OMM) and oculofacial-skeletal myorhythmia (OSFM) : pathognomic of Whipple's disease
- CNS involvement usually has poor prognosis. [1] [2]
Ocular involvement : rare and can involve: [2]
- uveitis,
- retinitis ,
- vitritis
- retrobulbar neuritis
- papilledema .
- Ocular involvement is often seen in patients with CNS disease. [2]
Skeletal muscle involvement
Cardiac involvement :may have varied presentation .[1] [2]
- pericarditis
- myocarditis
- heart failure
- sudden Cardiac death
- Endocarditis : associated with negative blood cultures. Endocarditis is often preceded by arthritis and arthralgias .[1] [2]
Pulmonary involvement [2]
- manifest as pleuritic chest pain
- non productive cough
- dyspnea
- Chest x-ray often shows pleural effusion , pulmonary infiltration, or granulomatous mediastinal adenopathy. Often abdominal lymph nodes may be involved.
Hematological finding
Cutaneous finding
- melanoderma : less frequently reported these days as Whipple's disease is increasingly recognized early. [1]
Renal involvement : late in course, may be seen rarely. [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 2.5 2.6 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.