A chronic multisystemic disease caused by the gram-positive bacterium Tropheryma whipplei, first described by G.H. Whipple in 1907.
Affected patients are typically middle-aged white men, who may present with weight loss, arthralgia, diarrhoea, and fever.
The infection is very rare, even though T whipplei is ubiquitously present in the environment.
Diagnosis is mainly based on duodenal biopsies. Histological findings are characterised by foamy macrophages in the lamina propria that contain large amounts of diastase-resistant periodic acid-Schiff (PAS)-positive particles in the cytoplasm. Polymerase chain reaction (PCR)-based diagnosis can be established from cerebrospinal fluid (CSF) or synovial fluid.
Recommended treatment is with intravenous ceftriaxone for 14 days, followed by oral trimethoprim/sulfamethoxazole for 1 year.
Whipple's disease is a rare, multisystemic, chronic infectious disease caused by Tropheryma whipplei, a gram-positive bacterium from the Actinomycetes clade with a genome rich in guanine and cytosine (G-C) nucleotides.T whipplei is found ubiquitously in the environment and in healthy carriers. A genetic predisposition for Whipple's disease seems to enable the establishment of an infection. Patients are typically middle-aged white men, who present with arthralgia, weight loss, diarrhoea, and fever.
History and exam
Key diagnostic factors
- presence of risk factors
- weight loss
- supranuclear ophthalmoplegia
Other diagnostic factors
- abdominal pain
- skin darkening
- confusion, memory impairment, altered level of consciousness, or dementia
- anxiety, depression, hypomania, psychosis, change in personality
- myoclonic signs
- brisk reflexes, extensor plantar responses, weakness predominating in arm extensors and leg flexors, hypertonia
- amenorrhoea, polydipsia, hyperphagia, decreased libido
- oculomasticatory and oculofacioskeletal myorhythmias
- cranial nerve involvement
- extrapyramidal movement disorder
- peripheral neuropathies
- age >50 years
- male sex
- genetic factors
1st investigations to order
- serum albumin
- serum CRP
- serum ESR
- upper GI endoscopy
- Periodic acid-Schiff (PAS) staining of duodenal biopsies
- Tropheryma whipplei-specific immunohistochemistry
- electron microscopy
patients without CNS involvement
patients with CNS involvement
- Seronegative rheumatoid arthritis
- Coeliac disease
Diarrhoea in adults
Diarrhoea in childrenMore Patient leaflets
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