1. Etiology

• an infectious disease caused by a rod-shaped bacterium resulting in the widespread appearance of fat and glycoprotein-filled macrophages throughout the body
• the small intestinal mucosa in always severely affected and the lesions observed in the mucosa are specific and diagnostic
• virtual replacement of most of the cellular elements in the lamina propria by macrophages is specific to Whipples Disease

1. Gastrointestinal Manifestations

• chronic diarrhea
• malabsorption with steatorrhea
• abdominal distension/tenderness

2. Central Nervous System Manifestations

• confusion
• focal cranial nerve signs
• memory loss
• nystagmus
• ophthalmoplegia

3. Systemic Manifestations

• fever (low-grade) and weight loss
• cough, hilar adenopathy, pleuritic effusion/pain
• arthralgia & polyarthritis
• increased skin pigmentation, peripheral lymphadenopathy

1. For Malabsorption

• see file on "Malabsorptive Disorders"
• serum - anemia

2. Biopsy - Duodenum or Jejunum

• mucosal injury in the small intestine is characterized by
  • edematous mucosa with variable alterations in villus structure
  • accumulation of fat & glycoprotein-filled (PAS+) macrophages within the lamina propria
  • the presence of bacilli in the mucosa
• malabsorption is caused by this mucosal injury

1. Antibiotics

• Septra for at least 1 year
• Penicillin and Tetracycline are associated with a 40% relapse rate