Whipple Disease

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 22/04/2025

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 Distal duodenum and jejunum are most often involved, with distal small bowel/ileum involved in severe cases

image Thickened, irregular folds with sand-like micronodules
image Small bowel lumen may be normal or mildly dilated
• CT

image Low-density enlarged mesenteric and retroperitoneal lymph nodes that may have near fat-density
image Thickened proximal small bowel folds ± submucosal edema due to hypoalbuminemia
• MR

image Lymph nodes may show ↑ T1 signal due to fat

TOP DIFFERENTIAL DIAGNOSES

• Celiac disease
• Intestinal opportunistic infections
• Dysgammaglobulinemia
• Intestinal metastases and lymphoma

PATHOLOGY

• Caused by  Tropheryma whipplei  (probably orally acquired)
• Not all patients with  Tropheryma whipplei infection develop Whipple disease, and underlying autoimmune disorders or genetic abnormalities may potentiate clinical syndrome

CLINICAL ISSUES

• Mostly affects middle-aged Caucasian men
• Prodromal phase: Fevers, fatigue, arthralgias, arthritis
• Late phase: Diarrhea, malabsorption, steatorrhea, adenopathy, abdominal pain

image GI symptoms generally later manifestation of disease
• Can be fatal without therapy (long-term antibiotics)
• Clinical symptoms often subside quickly after therapy
image
(Left) Small bowel follow-through shows nodular thickening of the jejunal folds image in a patient with Whipple disease.

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(Right) Axial CECT shows jejunal fold thickening image, as well as low-attenuation mesenteric and periaortic lymphadenopathy image. Endoscopic biopsy of the jejunal mucosa confirmed PAS-positive macrophages containing gram-negative, acid-fast bacilli, characteristic of Whipple disease.
image
(Left) Small bowel follow-through in a 40-year-old man with arthralgias and diarrhea demonstrates thickened, nodular jejunal folds.

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(Right) Axial CECT in the same patient shows marked thickening of the small bowel image with retroperitoneal and mesenteric lymphadenopathy image, which appears lower in attenuation than in most other causes of adenopathy. Endoscopic biopsy of the jejunal mucosa revealed villi distended with macrophages full of periodic acid-Schiff-positive bacilli, diagnostic of Whipple disease.
image
Axial CECT shows very low-density nodes in the retroperitoneum and mesentery.

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Small bowel follow-through shows micronodular fold thickening of most of the small bowel but no excess fluid.
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Axial CECT shows mural thickening of long segment of jejunum.
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Small bowel follow-through shows the micronodular pattern of the jejunum in a 40-year-old man with Whipple disease.