C171 Whipple’s disease

Introduction

Systemic tissue damage characterized by macrophages loaded with Tropheryma whipplei; partially destroyed organisms are present in macrophage lysosomes (positive for PAS).

Etiologic agent

Epidemiology

Pathogenesis and pathology

Small bowel lamina propria

Macrophages compress lacteals.
Chylomicrons cannot be transferred from enterocytes to lymphatics.
Results in fat malabsorption and steatorrhea

Intense pink discoloration – PAS stain is particularly effective at highlighting polysaccharides of the fungal cell wall, mucosubstances secreted by epithelia, and basement membranes. 

Others

Synovium of joints (arthritis)
Cardiac valves
Lymph nodes
CNS.

Clinical manifestations

Asymptomatic colonization/carriage

Acute infection

Chronic infection

  • “Classic” Whipple’s disease
    • Neurologic disease
    • Cardiac disease
    • Pulmonary disease
    • Lymphatic disease
    • Ocular disease
    • Dermatologic disease
    • Miscellaneous sites
  • Isolated infection
  • Reinfection/relapsing disease/immune reconstitution inflammatory syndrome (IRIS)

Treatment

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