GASTROENTEROLOGY ARTICLE OF THE WEEK
March 3, 2005
Bai JC, Mazure RM, Vazques H, et al. Whipple’s Disease. Clin Gastroenterology Hepatol 2004;2:849-860
[WD = Whipple’s Disease]
1. Characteristic constellation of presenting symptoms in WD include
a. acute onset presentation of dementia, edema and heart murmur
b. weight loss, chronic diarrhea, fever and arthralgia
c. weight gain, colon polyps and constipation
d. headaches, weight gain and skin pigmentation
2. Typical neurologic presenting findings in WD include
a. dementia and inability to ambulate
b. Parkinson’s type rigidity
c. dementia, ophtahlmoplegia and myoclonus
d. Guillan-Barre syndrome
True or False
3. The presence of generalized lymphadenopathy should suggest lymphoma rather than WD.
4. WD occurs primarily in caucasian middle age males.
5. The typical diarrhea in WD is due to a secretory process in the small bowel, absorption of fat is typically normal
6. The Whipple bacillus is a member of the actinomycete line, it is easily cultured from small bowel biopsy samples
7. Ascites, hepatomegaly and splenomegaly in conjuction with an increased platelet count suggests WD instead of primary liver disease
8. CNS involvement in WD is often irreversible despite successful antibiotic therapy, in those who respond, neurologic relapse is frequent, even years after therapy
9. The classic histologic findings for WD in small bowel biopsy is the presence of foamy macrophages that stain PAS-positive, the villi are short and thickened
10. Malabsorption in WD is thought to be caused by lymphatic obstruction from lacteal compression in the lamina propria and lymph nodes .
11. The PCR test for the Whipple’s bacillus is highly specific, a single positive result is diagnostic for infection
12. A positive IgA antibody in serum against the WD bacterium is diagnostic of the disease
13. The most typical finding of WD on EGD is the presence of multiple whitish-yellow plaques diffusely distributed in the intestinal mucosa, but is not pathognomonic.
14. MAC infection of the duodenum causes similar histologic findings as WD, however, MAC is acid-fast positive, while the WD bacillus is not
15. In atypical cases, the recommended diagnostic test for WD in the small bowel is electron microscopy of sections that show typical findings on light microscopy.
16. The most recommended antibiotic regimen consists of Penicillin G IM and streptomycin IM or a third generation cephalosporin daily for 2 weeks followed by TMS for at least 1 year