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Whipple's disease
Classification and external resources

light microscopy of small intestine;Whipples Disease: an infiltrate of foamy macrophages is present in the lamina propria
ICD-10 K90.8
ICD-9 040.2
DiseasesDB 14124
MedlinePlus 000209
eMedicine med/2409 neuro/397
MeSH D008061

Whipple's disease is a rare, systemic infectious disease caused by the bacterium Tropheryma whipplei.[1] First described by George Hoyt Whipple in 1907[2][3] and commonly considered a gastrointestinal disorder, Whipple's disease primarily causes malabsorption but may affect any part of the body including the heart, lungs, brain, joints, and eyes. Weight loss, diarrhea, joint pain, and arthritis are common presenting symptoms, but the presentation can be highly variable and approximately 15% of patients do not have these classic signs and symptoms.[4] Whipple's disease is significantly more common in men, with 87% of patients being male.[5] When recognized and treated, Whipple's disease can usually be cured with long-term antibiotic therapy; untreated the disease is ultimately fatal.

Contents

Symptoms

Symptoms of Whipple's disease are:

  • Arthralgias or a migratory, non-deforming Arthritis occurs in 80% and is typically the first symptom experienced. It may be transient or chronic.
  • Malabsorption
  • Intestinal lipodystrophy (accumulation of fatty deposits in lymph nodes of the intestine)
  • Lymphadenopathy
  • Abdominal pain
  • Diarrhea
  • Fever
  • Melanoderma
  • Neurological symptoms
    • Cognitive changes
    • Nystagmus
    • Oculomasticatory myorhythmia (OMM) (pendular vergence oscillations of the eyes and synchronous contractions of the masticatory but not palatal muscles) is pathognomonic of Whipple disease.[6]

Diagnosis

Common clinical signs and symptoms of Whipple's disease include weight loss, skin rashes, diarrhea, joint pain or arthritis, fever, and adenopathy. Diagnosis is made by intestinal biopsy, which reveals presence of the organism as PAS-positive macrophage inclusions. Immunohistochemical staining for antibodies against T. whipplei has been used to detect the organism in a variety of tissues, and a confirmatory PCR-based assay is also available.

Endoscopy of the duodenum and jejunum can reveal pale yellow shaggy mucosa with erythematous eroded patches in patients with classic intestinal Whipple's disease, and small bowel X-rays may show some thickened folds.

Treatment

Treatment is with penicillin, ampicillin, tetracycline or co-trimoxazole for one to two years.[4] Any treatment lasting less than a year has an approximate relapse rate of 40%.[citation needed] In the January 4, 2007 issue of the New England Journal of Medicine, Fenollar et al. suggest the use of doxycycline with hydroxychloroquine for 12 to 18 months.[5] Sulfonamides (sulfadiazine or sulfamethoxazole) should be added for treatment of neurological symptoms.[5]

References

  1. ^ Puéchal X (March 2002). "Whipple's disease". Joint Bone Spine 69 (2): 133–40. doi:10.1016/S1297-319X(02)00359-7. PMID 12027303. 
  2. ^ synd/3892 at Who Named It?
  3. ^ Whipple GH (1907). "A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acid in the intestinal and mesenteric lymphatic tissues". Bull Johns Hopkins Hosp 18: 382–93. 
  4. ^ a b Bai J, Mazure R, Vazquez H, Niveloni S, Smecuol E, Pedreira S, Mauriño E (October 2004). "Whipple's disease". Clinical Gastroenterology and Hepatology 2 (10): 849–60. doi:10.1016/S1542-3565(04)00387-8. PMID 15476147. 
  5. ^ a b c Fenollar F, Puéchal X, Raoult D (January 2007). "Whipple's disease". The New England Journal of Medicine 356 (1): 55–66. doi:10.1056/NEJMra062477. PMID 17202456. 
  6. ^ Schwartz MA, Selhorst JB, Ochs AL, Beck RW, Campbell WW, Harris JK, Waters B, Velasco ME (December 1986). "Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease". Annals of Neurology 20 (6): 677–83. doi:10.1002/ana.410200605. PMID 2434019. 

External links

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