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

Epidemiology

Etiology

Somehow related to autoimmune process

Mnemonic

PAS the foamy Whipped cream in a CAN.

A multisystemic disease, often with a long prodromal phase.
Classic Tetrad (CAN):
- Cardiac: Endocarditis (often culture-negative), pericarditis, heart failure.
- Arthralgias: Migratory, non-deforming polyarthralgias are often the earliest symptom, preceding GI symptoms by years.
- Neurologic: Dementia, memory loss, confusion, and pathognomonic oculomasticatory myorhythmia (pendular eye movements with concurrent jaw contractions).
GI Manifestations:
- Malabsorption leading to steatorrhea (foul-smelling, fatty stools), chronic diarrhea, and abdominal pain.
- Significant weight loss is a hallmark feature.
Other common findings:
- Generalized lymphadenopathy.
- Fever.
- Hyperpigmentation of the skin, especially in sun-exposed areas.

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