Internal Medicine

0521779407-23 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:24

Whipple’s Disease 1553

■Maldigestion due to pancreatic insufficiency (chronic pancreatitis)

or pancreatic duct obstruction (pancreatic carcinoma): fecal fat ele-

vated but xylose absorption normal

■Irritable bowel syndrome (multiple stools but scanty quantity and

no malabsorption)

management

What to Do First

■If intestinal biopsy reveals PAS-positive macrophages and positive

PCR for T. Whippelii, then CSF fluid should be obtained for PCR also

General Measures

■Add vitamin supplements for 2–3 months (multivitamins, folic acid,

fat-soluble vitamins [A,D,E])

■Initiation of appropriate long-term antibiotic therapy (see below)

specific therapy

■Long-term treatment with oral antibiotics (trimethoprim 160 mg/

sulfamethoxazole 800 mg p.o. b.i.d. for 1 to 2 years)

■If cerebral involvement, parenteral Penicillin G 1.2 million units and

streptomycin 1 Gm daily should be given initially for 14 days, fol-

lowed by the oral drugs

follow-up

■Response to antibiotic therapy usually prompt, within a week or so

■Monitoring of improvement in functional absorptive parameters

(xylose, fecal fat); serum albumen, correction of anemia

■Repeat intestinal biopsy for histology and PCR analysis for T. whip-

pelii at 6–12 months

■Long-term monitoring: yearly assessment of nutrition, cardiac and

neurologic status

■Prompt thorough evaluation at any suggestion of enteric, cardiac or

neurologic recurrence over the years

complications and prognosis

■At least 90% of patients have a complete and sustained response to

antibiotic therapy

■Relapse rate 15–20% in earlier years when oral penicillin was

most widely used; now relapse much lower after trimethoprim/

sulfamethoxazole therapy

■CNS recurrence may be severe and extensive, mimicking more com-

mon diseases such as ischemia, stroke or dementia