Whipple's Disease
Article Outline
A 51-year-old man presented with migratory arthritis involving the knees, wrists, and elbows and weight loss of 15 kg during a 12-month period. The patient was treated with a combination of NSAIDs and steroids. Two months before presentation he developed intermittent diarrhea. On esophagogastroduodenoscopy (EGD), the mucosa was slightly edematous. With magnification endoscopy (115×, Olympus Q160Z; Olympus Europe, Hamburg, Germany) the duodenal villi appeared edematous, slightly flattened, and engorged, and some of these were filled with a white material (Figure A). Duodenal biopsy specimens revealed “foamy” macrophages with intracellular granules The periodic acid–Schiff (PAS) stain was positive (original magnification, 200×) (Figure B). Quantitative real-time polymerase chain reaction (PCR) (LightCycler; Roche, Mannheim, Germany) was positive for Whipple's disease (WD). The patient was treated for 2 weeks with intravenous ceftriaxone followed by trimethoprim-sulfamethoxazole (TMP-SMX) for 12 months, with improvement of symptoms. WD is a rare multisystemic chronic bacterial infection that can affect various systems, including musculoskeletal, gastrointestinal, neurologic, and cardiovascular.1 The etiologic agent of WD is the bacteria Tropheryma whipplei (formerly T whippelii).1, 2 There are very few studies documenting the endoscopic findings and pictures of WD. Endoscopic findings include mucosal erythema, edema, enlarged duodenal folds, and lymphangiectasias. In our patient we found magnification endoscopy useful to characterize the structure of the villi, which appeared engorged, flattened, and filled with white contents, which likely represented lymph and/or debris of bacteria. The diagnosis of WD can be very difficult.1, 2 Laboratory abnormalities in WD are variable and reflect the major organs involved. Nonetheless, most patients will exhibit an increased inflammatory reaction such as elevated sedimentation rate and thrombocytosis.1 Other laboratory and radiologic abnormalities will depend on the system involved.1 The basic principle to diagnose WD is the demonstration of the bacillus in any tissue biopsy by using standard hematoxylin-eosin and PAS stains and PCR.2, 3 The most common approach to obtain tissue in these patients is an EGD with small bowel biopsies.1, 2, 3 The most important aspect to consider when treating patients with WD is to use antibiotics that cross the blood-brain barrier, because central nervous system involvement is common, and most relapses of WD occur within the central nervous system.1 TMP-SMX crosses the blood-brain barrier, whereas tetracycline, another effective antibiotic to treat WD, does not cross the blood-brain barrier.1 All patients should receive an initial 2-week course of intravenous antibiotics before embarking on long-term therapy. Currently, the recommendation is to initiate therapy with an intravenous cephalosporin (ie, cefrtriaxone) or meropenem followed by long-term TMP-SMX.1, 2
References
- . Whipple's disease. Lancet. 2003;361:239–246
- . Diagnosis and therapy monitoring of Whipple's arthritis by polymerase chain reaction. Rheumatology. 2000;39:1427–1428
- . Diagnostic application of a polymerase chain reaction assay for the Whipple's disease bacterium to intestinal biopsies. Gastroenterology. 1996;110:1735–1743
PII: S1542-3565(08)00205-X
doi:10.1016/j.cgh.2008.02.042
© 2008 AGA Institute. Published by Elsevier Inc. All rights reserved.
