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Whipple's Disease

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A chronic, relapsing multisystem disease first described by GH Whipple, an American pathologist, in 1907.1 He described the disease as an intestinal lipodystrophy characterised by:

  • Weight loss
  • Chronic cough
  • Fat accumulation in the intestine, mesenteric lymph nodes and stool

It is now thought to be due to infection with actinomycete Tropheryma whippelii combined with defective cell-mediated immunity.2 It is probably acquired as an enteric infection as this organism is commonly found in sewage effluent.

Traditionally, Whipple's disease has presented a diagnostic challenge to both clinicians and pathologists.3,4

Epidemiology2,5

This is a rare condition. Incidence is estimated as less than 1 per 1,000,000 per annum.

Risk factors

Incidence is increased in:

  • Middle-age and older
  • Males>females
  • Caucasian patients
  • Family clusters (suggesting an immunogenetic component)
  • HLA-B27 haplotype
Presentation3,5

Many different forms of presentation with the common ones being:

  • Polyarthralgia - transient and episodic (often a prodromal symptom)
  • GI symptoms:
    • Abdominal pain
    • Diarrhoea
    • Distension
    • Flatulence
    • Steatorrhea (due to malabsorption)
  • Anorexia and weight loss
  • Intermittent low-grade fever
  • Chronic cough
  • Hyperpigmentation (occurs in 50%)

May also be:

  • Generalised lymphadenopathy
  • Anaemia and more rarely clotting abnormalities
  • Cardiac involvement - pericarditis, myocarditis, valve lesions
  • CNS involvement in 10% - dementia, ophthalmoplegia, myoclonus, oculomasticatory movements (convergent eye movements with simultaneous chewing movements), gait abnormalities, seizures, coma
  • Ocular involvement - uveitis, vitritis, keratitis, retinitis, retinal haemorrhages
  • Pulmonary involvement - pleural effusion, mediastinal widening (due to lymphadenopathy)
  • Protein-losing enteropathy with hypoalbuminaemia and oedema (due to protein loss)
Differential diagnosis
Investigations6

Diagnosis requires a high index of clinical suspicion:

  • Routine bloods and malabsorption tests are non-specific.
  • Imaging will confirm involvement of different organs but is not diagnostic.
  • Biopsy of affected tissue, usually the duodenum, shows infiltration of the laminae propria with PAS(periodic acid Schiff stain)-positive macrophages with intracellular clumps of T.Whippelii. This test is negative in about 30%, more commonly those without GI involvement and biopsy of other organs or lymph nodes may be indicated.
  • PCR of bacterial RNA (unique 16s rRNA sequence) is increasingly used in diagnosis and can be done from peripheral blood, CSF and other tissue samples.
Management7
  • Oral Co-trimoxazole for at least one year or, if allergic, ceftriaxone or chloramphenicol.
  • Some have argued for an alternative regimen - long-term doxycycline and hydroxychloroquine in patients without neurological involvement, adding high dose of sulfamethoxazole or sulfadiazine only in those with neurological involvement.8
  • Repeat PCR at the end of treatment.
  • With neurological involvement, treatment often commences with IV cephalosporin and aminoglycoside.
Prognosis
  • Insidious progression and fatal if untreated
  • Locomotor and GI symptoms may improve very rapidly with treatment but histological remission can take several years.
  • Follow up closely for signs of recurrence - relapse in about 35%.


Document references
  1. Fenollar F, Puechal X, Raoult D; Whipple's disease. N Engl J Med. 2007 Jan 4;356(1):55-66.
  2. Desnues B, Ihrig M, Raoult D, et al; Whipple's disease: a macrophage disease.; Clin Vaccine Immunol. 2006 Feb;13(2):170-8.
  3. Rakshit RC, Mackay JD; A diagnostic conundrum.; Postgrad Med J. 2003 Sep;79(935):540, 545-6.
  4. Mahnel R, Marth T; Progress, problems, and perspectives in diagnosis and treatment of Whipple's disease.; Clin Exp Med. 2004 Sep;4(1):39-43. [abstract]
  5. Oxford textbook of medicine, 4th edition. Ed. D. Warrel et al. OUP Feb 2004 ISBN 0198529988
  6. Marth T, Raoult D; Whipple's disease.; Lancet. 2003 Jan 18;361(9353):239-46. [abstract]
  7. Buckle MJ, Ellis RW, Bone M, et al; Neurologically presenting Whipple disease: case report and review of the literature. J Clin Pathol. 2008 Oct;61(10):1140-1. Epub 2008 Apr 1. [abstract]
  8. Knaapen HK, Barrera P; Therapy for Whipple's disease. J Antimicrob Chemother. 2007 Sep;60(3):457-8. Epub 2007 Jul 10. [abstract]

Internet and further reading Acknowledgements EMIS is grateful to Dr Chloe Borton for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2008.
DocID: 846
Document Version: 21
DocRef: bgp1300
Last Updated: 27 Nov 2008
Review Date: 27 Nov 2010

The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest. Find out more about updating.

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