Whipple's disease is caused by Tropheryma whippelei, a Gram positive bacterium.
The typical presentation is of a wasting illness with arthralgia, arthritis, fever, and diarrhoea. Malabsorption is common.
If the disease affects the small intestine, patients may also have steatorrhea.
The typical presentation is of a wasting illness with arthralgia, arthritis, fever, and diarrhoea. Malabsorption is common.
If the disease affects the small intestine, patients may also have steatorrhea.
Early symptoms include fever, malaise, and lymphadenopathy may precede the onset of GI symptoms by 1-10 years.
The clinical manifestations of the disease are thought to be caused by infiltration of tissues by T. whippelei and the uptake of these organisms into tissue macrophages.
The CNS and cardiovascular system may also be involved.
The clinical manifestations of the disease are thought to be caused by infiltration of tissues by T. whippelei and the uptake of these organisms into tissue macrophages.
The CNS and cardiovascular system may also be involved.
Many of the physical signs in Whipple's disease are due to malabsorption:
- cachexia
- abdominal distention
- glossitis
- angular cheilitis
- Chvostek's or Trousseau's signs (secondary to hypocalcaemia)
- gingivitis (secondary to vitamin C deficiency)
- night blindness (secondary to vitamin A deficiency).
There may be perioral and malar hyperpigmentation.
Depending on the site of involvement, patients with CNS disease may develop meningoencephalitis, ataxia and clonus (cerebellum) or loss of inhibition (frontal).
Cardiac complications include pericarditis, myocarditis, and left-heart valve lesions. The patient may have finger clubbing.
Depending on the site of involvement, patients with CNS disease may develop meningoencephalitis, ataxia and clonus (cerebellum) or loss of inhibition (frontal).
Cardiac complications include pericarditis, myocarditis, and left-heart valve lesions. The patient may have finger clubbing.
Whipple's disease is most common in white males aged 40-50 years and rarely is described in women (M:F ratio 9:1).
There is an association with the HLA-B27 haplotype.
The diagnosis is made by demonstrating the presence of T. whippelei DNA in tissue by PCR.
There is an association with the HLA-B27 haplotype.
The diagnosis is made by demonstrating the presence of T. whippelei DNA in tissue by PCR.
Treatment is with an initial two-week course of parenteral penicillin and streptomycin; followed by a prolonged course (one year) of tetracycline.
Untreated, patients have a poor prognosis.
Untreated, patients have a poor prognosis.
Tips: think whipple if :
Malabsorption plus:
Arthritis
Neurological symptoms
Cardiac problems
Middle age male patient
Based on these clinical observations, the current recommendation for treatment of Whipple’s disease is to begin with an induction phase using either penicillin G plus streptomycin or a third-generation cephalosporin, such as ceftriaxone, followed by treatment with at least one drug that efficiently crosses the blood-brain barrier (e.g., trimethoprim/sulfamethoxazole) for at least one year.
ReplyDeletePolyarthralgias of the knees, ankles, elbows, or fingers; uveitis; weight loss; diarrhea; abdominal pain; and skin hyperpigmentation are all associated with which disease?
ReplyDelete1-Whipple disease
2-Haemochromatosis
3-IBS
4-AAT def
These are all clinical manifestations of Whipple’s disease. Arthralgias can precede intestinal symptoms by several years. Polyarthralgias are a common symptom. Skin hyperpigmentation can be found in 17% to 66% of patients. Dominant signs of Whipple’s disease include diarrhea, weight loss, and abdominal pain.
The histopathologic features of intestinal Whipple’s disease are quite distinctive. On gross inspection, the mucosa of the distal duodenum and jejunum is abnormal in most patients. Whitish to yellow plaque-like patches are observed in approximately three fourths of patients; alternatively, the mucosa may appear pale yellow.
ReplyDeleteAlmost all patients with Whipple’s disease have involvement of the intestinal tract by this infection, regardless of whether gastrointestinal symptoms are present. Thus, the primary diagnostic approach to a patient with clinically suspected Whipple’s disease is upper endoscopy with mucosal biopsy. To avoid sampling errors in patients with patchy lesions, one should obtain approximately five biopsy specimens from regions as far distal as possible within the small intestine. Histologic examination with routine hematoxylin and eosin and PAS stains is usually sufficient to reach a diagnosis. In some cases, findings may be corroborated with silver stains; in contrast, the Gram stain is less useful in this infection. Traditionally, electron microscopy has been used as the gold standard for confirming the diagnosis of Whipple’s disease. Currently, polymerase chain reaction analysis serves in this capacity.
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