Whipple Disease (DD)

• Definition: an infectious disease caused by Tropheryma whipplei, an intracellular gram-positive bacteria

• Epidemiology

  • Very rare

  • Most commonly occurs in males older than 40 years

• Intestinal manifestations

  • Abdominal pain

  • Malabsorption syndrome (including diarrhea and/or steatorrhea): commonly occurs later in the disease progression

• Extraintestinal manifestations

  • Enteropathic arthritis (60% of cases)

  • Arthralgias and arthritis, especially sacroiliitis (40% of cases)

  • Fever

  • Hyperpigmentation affecting sun-exposed areas

  • Polyserositis

  • Lymphadenopathy

  • Cardiac symptoms (e.g., valve insufficiencies)

  • Neurological symptoms (e.g., myoclonia, ataxia, impairment of oculomotor function)

• Small intestine biopsies: detection of PAS-positive foamy macrophages in the lamina propria

  • If gastrointestinal symptoms are absent, biopsies may also be taken from other sites with disease activity

• PCR testing and immunohistochemistry staining

• Imaging may show enlarged mesenteric nodes.

• If neurological complaints occur: Perform a lumbar puncture and CSF analysis and neuroimaging (MRI).

• IV ceftriaxone for 2 weeks

• Maintenance treatment with oral trimethoprim-sulfamethoxazole for 1 year

Anyone who CANT appreciate the foamy, PAStoral rivers of England gets Whipped: the most important features of Whipple disease are Cardiac symptoms, Arthralgias, Neurologic symptoms, Trots (diarrhea), and foamy, PAS-positive macrophages on biopsy.

Whipple disease is lethal if left untreated!