Whipple's disease pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Overview

Whipple’s disease is a very rare disease. Therefore, some aspects of pathogenesis have remained unclear. Tropheryma whipplei is usually transmitted through oral route to human hosts. There is no known causative genetic factor for Whipple's disease. However, genetic and immunologic factors play important roles on clinical manifestation of T. whipplei infection.

Pathophysiology

Pathogenesis

  • The bacterium lives in soil and wastewater. Farmers and everyone who has any contact with contaminated soil and water are at high risk of the infection.[3]
  • It is transmitted through oro-oral and feco-oral routes. The poor sanitation is associated with Tropheryma whipplei infection.[4]
  • It is believed that human being is the only host for this bacterium.[5]
  • Tropheryma whipplei infection causes four different clinical manifestations: acute infection, asymptomatic carrier state, the classic Whipple’s disease, and localized chronic infection.[9][10]
 
 
 
 
 
 
 
 
Contamination via oro-oral or feco-oral route
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Acute infection
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Antibodies production
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Strong immune response
 
 
 
 
 
Insufficient immune response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Complete eradication
 
 
 
Chronic carrier
 
 
Chronic infection
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Classic Whipple's disease
 
 
 
Localized infection
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Cure
 
Relapse
 
Re-infection
 
Death
 

Immunologic response

Followings are some of the observations that indicate the immunologic nature of the Whipple's disease:

Defective cellular immunity

Defective humoral immunity

Genetics

There is no known causative genetic factor for Whipple's disease. However, there is an association between Whipple's disease and some immunologic defects.

  • Studies showed that individuals with specific HLA type (HLA alleles DRB1*13 and DQB1*06) have a higher risk of Whipple's disease.[9]

Associated Conditions

The most important conditions associated with Whipple's disease include:

Gross Pathology

  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of Whipple's disease.

Microscopic Pathology

Below images show the characteristic feature of Whipple's disease. foamy macrophages are present in the lamina propria.[18]

References

  1. Schneider T, Moos V, Loddenkemper C, Marth T, Fenollar F, Raoult D (2008). "Whipple's disease: new aspects of pathogenesis and treatment". Lancet Infect Dis. 8 (3): 179–90. doi:10.1016/S1473-3099(08)70042-2. PMID 18291339.
  2. Schwartzman, Sergio; Schwartzman, Monica (2013). "Whipple's Disease". Rheumatic Disease Clinics of North America. 39 (2): 313–321. doi:10.1016/j.rdc.2013.03.005. ISSN 0889-857X.
  3. Keita, Alpha Kabinet; Diatta, Georges; Ratmanov, Pavel; Bassene, Hubert; Raoult, Didier; Roucher, Clémentine; Fenollar, Florence; Sokhna, Cheikh; Tall, Adama; Trape, Jean-François; Mediannikov, Oleg (2013). "Looking for Tropheryma whipplei Source and Reservoir in Rural Senegal". The American Journal of Tropical Medicine and Hygiene. 88 (2): 339–343. doi:10.4269/ajtmh.2012.12-0614. ISSN 0002-9637.
  4. Keita, Alpha Kabinet; Brouqui, Philippe; Badiaga, Sékéné; Benkouiten, Samir; Ratmanov, Pavel; Raoult, Didier; Fenollar, Florence (2013). "Tropheryma whipplei prevalence strongly suggests human transmission in homeless shelters". International Journal of Infectious Diseases. 17 (1): e67–e68. doi:10.1016/j.ijid.2012.05.1033. ISSN 1201-9712.
  5. Marth, Thomas; Moos, Verena; Müller, Christian; Biagi, Federico; Schneider, Thomas (2016). "Tropheryma whipplei infection and Whipple's disease". The Lancet Infectious Diseases. 16 (3): e13–e22. doi:10.1016/S1473-3099(15)00537-X. ISSN 1473-3099.
  6. 6.0 6.1 Marth T, Strober W (1996). "Whipple's disease". Semin. Gastrointest. Dis. 7 (1): 41–8. PMID 8903578.
  7. Dolmans, Ruben A. V.; Boel, C. H. Edwin; Lacle, Miangela M.; Kusters, Johannes G. (2017). "Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections". Clinical Microbiology Reviews. 30 (2): 529–555. doi:10.1128/CMR.00033-16. ISSN 0893-8512.
  8. 8.0 8.1 8.2 8.3 8.4 Marth T, Roux M, von Herbay A, Meuer SC, Feurle GE (1994). "Persistent reduction of complement receptor 3 alpha-chain expressing mononuclear blood cells and transient inhibitory serum factors in Whipple's disease". Clin. Immunol. Immunopathol. 72 (2): 217–26. PMID 7519533.
  9. 9.0 9.1 Marth, Thomas (2009). "New Insights into Whipple's Disease – A Rare Intestinal Inflammatory Disorder". Digestive Diseases. 27 (4): 494–501. doi:10.1159/000233288. ISSN 1421-9875.
  10. Street, Sara; Donoghue, Helen D; Neild, GH (1999). "Tropheryma whippelii DNA in saliva of healthy people". The Lancet. 354 (9185): 1178–1179. doi:10.1016/S0140-6736(99)03065-2. ISSN 0140-6736.
  11. 11.0 11.1 11.2 Marth T, Kleen N, Stallmach A, Ring S, Aziz S, Schmidt C, Strober W, Zeitz M, Schneider T (2002). "Dysregulated peripheral and mucosal Th1/Th2 response in Whipple's disease". Gastroenterology. 123 (5): 1468–77. PMID 12404221.
  12. Schinnerling K, Moos V, Geelhaar A, Allers K, Loddenkemper C, Friebel J, Conrad K, Kühl AA, Erben U, Schneider T (2011). "Regulatory T cells in patients with Whipple's disease". J. Immunol. 187 (8): 4061–7. doi:10.4049/jimmunol.1101349. PMID 21918190.
  13. 13.0 13.1 Ectors N, Geboes K, De Vos R, Heidbuchel H, Rutgeerts P, Desmet V, Vantrappen G (1992). "Whipple's disease: a histological, immunocytochemical and electronmicroscopic study of the immune response in the small intestinal mucosa". Histopathology. 21 (1): 1–12. PMID 1378814.
  14. 14.0 14.1 14.2 Marth T, Neurath M, Cuccherini BA, Strober W (1997). "Defects of monocyte interleukin 12 production and humoral immunity in Whipple's disease". Gastroenterology. 113 (2): 442–8. PMID 9247462.
  15. Schneider T, Stallmach A, von Herbay A, Marth T, Strober W, Zeitz M (1998). "Treatment of refractory Whipple disease with interferon-gamma". Ann. Intern. Med. 129 (11): 875–7. PMID 9867729.
  16. Desnues B, Raoult D, Mege JL (2005). "IL-16 is critical for Tropheryma whipplei replication in Whipple's disease". J. Immunol. 175 (7): 4575–82. PMID 16177102.
  17. Schneider, Thomas; Moos, Verena; Loddenkemper, Christoph; Marth, Thomas; Fenollar, Florence; Raoult, Didier (2008). "Whipple's disease: new aspects of pathogenesis and treatment". The Lancet Infectious Diseases. 8 (3): 179–190. doi:10.1016/S1473-3099(08)70042-2. ISSN 1473-3099.
  18. https://commons.wikimedia.org

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