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

Epidemiology

Etiology

Somehow related to autoimmune process

Tip

Cardiac symptoms, Arthralgias, and Neurologic symptoms are common.

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)

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.

Tip

AAT deficiency also has PAS-positive