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]; Sadaf Sharfaei M.D.[3]
Overview
Patients with Whipple's disease have various presentations. Most common symptoms of the classic Whipple's disease include joint pain, weight loss, diarrhea, and arthropathy. 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
Whipple's disease is a systemic disease that involves multiple organs. Many of the patients present with gastrointestinal symptoms, while others presents with localized involvement of different organs.
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, such as knees, elbows, wrists, and shoulders
- Multiple joint involvement
Less Common Symptoms
Central nervous system
- Asymptomatic involvement with detection of Tropheryma whipplei DNA in CSF: The most common finding
- Focal CNS involvement:
- Dementia
- Cerebral ataxia
- Hemiparesis
- Personality changes
- Opthalmoplegia
- Nystagmus
- Supranuclear opthalmoplegia (50% of patients)
- Myoclonus (25% of patients)
- Hypothalamic involvement (rarely)
- Polydipsia
- Hyperphagia
- Change in libido
- Change in sleep wake cycle
- Eye movement abnormalities (pathognomic)
- Oculomasticatory myorhythmia (OMM)
- Oculofacial-skeletal myorhythmia (OSFM)
- CNS involvement usually has poor prognosis.[1][2]
Ocular involvement
Ocular involvement is 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
- Pericarditis
- Myocarditis
- Congestive heart failure
- Sudden cardiac death
- Endocarditis associated with negative blood cultures. Endocarditis is often preceded by arthritis and arthralgias.[1] [2]
Pulmonary involvement
- Pleuritic chest pain
- Chronic cough
- Dyspnea
- Chest x-ray often shows pleural effusion , pulmonary infiltration, or granulomatous mediastinal adenopathy. Often abdominal lymph nodes may be involved.
Cutaneous finding
- Melanoderma: less frequently reported these days as Whipple's disease is increasingly recognized early.[1]
Renal involvement
Renal involvement is late in course, may be seen rarely.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 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 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.