Updated: 12/15/2021

Whipple Disease

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  • Snapshot
    • A 60-year-old Caucasian man presents to the emergency room with his family for fever and recent onset of dementia. His MMSE score was 20 at his last appointment with his primary care provider. His past medical history includes chronic diarrhea of unknown origin and nonspecific arthritis. On physical exam, he has multiple hyperpigmented macules on his skin, as well as a new murmur. His physician is suspicious of endocarditis.
  • Introduction
    • Malabsorption syndrome with multi-organ involvement
    • Caused by infection with Tropheryma whipplei
      • gram-positive bacillus
      • non acid-fast
      • Actinomyces spp. family
      • found in soil
  • Epidemiology
    • More commonly in
      • older patients (40s-50s)
      • Caucasian
      • male > female
    • Risk factors
      • previous exposure to soil or animals
      • immunodeficiency potentially predisposes to active disease (vs carrier state)
  • Presentation
    • Symptoms
      • CAN (though may not present all at once) plus diarrhea
        • Cardiac symptoms
          • endocarditis
          • myocarditis
          • pericarditis
        • Arthralgias (very common and early in disease)
        • Neurologic symptoms
          • dementia
          • seizures
          • encephalitis
          • 20% of patients with pathognomonic signs
            • oculomasticatory myorhythmia
            • oculo-facial-skeletal myorhythmia
      • weight loss
      • emaciation
      • chronic diarrhea
      • oligo-arthritis
    • Physical exam
      • fever
      • mesenteric lymphadenopathy
      • gray-brown pigmentation of skin
        • malabsorption of vitamin D
        • → hyperPTH
        • → increased melanocyte stimulating hormone
  • Evaluation
    • Endoscopic small bowel biopsy – most accurate test
      • periodic acid-Schiff (PAS)-positive foamy macrophages
      • intestinal lamina propria
    • Stool PCR may reflect carrier state – not definitive
    • CSF typically normal but should be performed
  • Differential Diagnosis
    • Celiac disease
    • Crohn disease
    • Lyme disease (arthralgias)
  • Treatment
    • Whipple without cardiac involvement
      • ceftriaxone or penicillin G for 2 weeks
      • followed by TMP-SMX for one year
    • Whipple with endocarditis
      • ceftriaxone or PCN G for 4 weeks
      • followed by TMP-SMX for one year
  • Complications
    • Jarisch-Herxheimer reaction to antibiotics
      • fevers
      • rigors
      • hypotension
    • Immune reconstitution inflammatory syndrome (IRIS) – up to 10%
      • high fever
      • mimics worsening disease but is actually a result of successful therapy
  • Prognosis
    • Persistence after treatment common
    • 30-50% rate of relapse
    • Fatal if antibiotics not given
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