Whipple Disease

Whipple disease is a rare, multivisceral chronic disease. It is common in Caucasian men, particularly farmers with occupational exposure to soil or animals. The overall incidence of Whipple disease is about one to three in one million people.

Last Updated: February 23, 2024

Whipple disease is caused by Tropheryma whipplei, a gram-positive bacteria. Symptoms associated with this infection are due to the accumulation of bacteria-laden macrophages within the small intestinal lamina propria and the mesenteric lymph nodes. The resulting lymphatic obstruction and impaired lymph damage lead to malabsorptive diarrhea.

The main clinical presentation of Whipple disease is a triad of diarrhea, weight loss, and arthralgia. Extraintestinal symptoms may precede malabsorption by months or years. These symptoms include:

  • Arthritis
  • Peripheral edema
  • Fever
  • Lymphadenopathy
  • Abdominal pain
  • Neurologic, cardiac, or pulmonary disease

The diagnosis of Whipple disease is done via a biopsy of the intestine and identification of T. whipplei in samples. A positive result for PAS-positive foamy macrophages in the small bowel biopsy is required in the current diagnostic criteria. Diagnosis can also be made if a positive result is concluded from a PCR test or immunohistochemical staining of bacterial antibodies. 

 

Antibiotic therapy is the main treatment for Whipple disease. The recommended regimen is an initial treatment with Ceftriaxone two grams daily or Penicillin G two million units every four hours, lasting for 2 to 4 weeks. This is followed by long-term treatment with sulfamethoxazole-trimethoprim, twice a day for at least 1 year to prevent relapse. Meropenem can also be used for the initial phase in patients with penicillin allergy.

Occupation and poor living conditions are risk factors for infection with T. whipplei. Exposure to unsanitary conditions, human feces, contaminated soil and water are associated with Whipple disease prevalence. Thus, appropriate protective equipment, such as gloves and masks is advised. Good personal hygiene should also be followed to prevent infection.



References:

 

Antunes C, Singhal M. Whipple Disease. [Updated 2022 Jul 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441937/

 

Keita AK, Mediannikov O, Ratmanov P, Diatta G, Bassene H, Roucher C, Tall A, Sokhna C, Trape JF, Raoult D, Fenollar F. Looking for Tropheryma whipplei source and reservoir in rural Senegal. Am J Trop Med Hyg. 2013 Feb;88(2):339-43. doi: 10.4269/ajtmh.2012.12-0614. 

 

Kumar, V., Abbas, A. K., & Aster, J. C. (2020). Robbins Basic Pathology (10th ed.). Elsevier - Health Sciences Division.

Last Updated: February 23, 2024