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

Epidemiology

Etiology

Pathophysiology

Somehow related to autoimmune process

Clinical features

• 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)
  • Migrating 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)

Diagnostics

• Small intestine biopsies: detection of PAS-positive foamy macrophages in the lamina propria
  • The purple highlights whipplei organisms in macrophages
• Imaging may show enlarged mesenteric nodes.

Treatment

• IV ceftriaxone for 2 weeks
• Maintenance treatment with oral trimethoprim-sulfamethoxazole for 1 year