Whipple's disease

Whipple’s disease is a chronic multisystemic disease caused by the gram-positive bacterium Tropheryma whipplei, first described by G.H. Whipple in 1907.

Affected patients are typically middle-aged white men, who may present with weight loss, arthralgia, diarrhoea, and fever.

The infection is very rare, even though T whipplei is ubiquitously present in the environment.

Diagnosis is mainly based on duodenal biopsies. Histological findings are characterised by foamy macrophages in the lamina propria that contain large amounts of diastase-resistant periodic acid-Schiff (PAS)-positive particles in the cytoplasm. Polymerase chain reaction (PCR)-based diagnosis can be established from cerebrospinal fluid (CSF) or synovial fluid.

Recommended treatment is with intravenous ceftriaxone for 14 days, followed by oral trimethoprim/sulfamethoxazole for 1 year.

Whipple's disease is a rare, multisystemic, chronic infectious disease caused by Tropheryma whipplei, a gram-positive bacterium from the Actinomycetes clade with a genome rich in guanine and cytosine (G-C) nucleotides.[1]Raoult D, Ogata H, Audic S, et al. Tropheryma whipplei twist: a human pathogenic Actinobacteria with a reduced genome. Genome Res. 2003 Aug;13(8):1800-9. http://genome.cshlp.org/content/13/8/1800.long http://www.ncbi.nlm.nih.gov/pubmed/12902375?tool=bestpractice.com [2]Bentley SD, Maiwald M, Murphy LD, et al. Sequencing and analysis of the genome of the Whipple's disease bacterium Tropheryma whipplei. Lancet. 2003 Feb 22;361(9358):637-44. http://www.ncbi.nlm.nih.gov/pubmed/12606174?tool=bestpractice.com T whipplei is found ubiquitously in the environment and in healthy carriers.[3]Schoniger-Hekele M, Petermann D, Weber B, et al. Tropheryma whipplei in the environment: survey of sewage plant influxes and sewage plant workers. Appl Environ Microbiol. 2007 Mar;73(6):2033-5. http://aem.asm.org/cgi/content/full/73/6/2033 http://www.ncbi.nlm.nih.gov/pubmed/17277223?tool=bestpractice.com [4]Maiwald M, Schuhmacher F, Ditton HJ, et al. Environmental occurrence of the Whipple's disease bacterium (Tropheryma whippelii). Appl Environ Microbiol. 1998 Feb;64(2):760-2. http://aem.asm.org/cgi/content/full/64/2/760 http://www.ncbi.nlm.nih.gov/pubmed/9464419?tool=bestpractice.com [5]Fenollar F, Trani M, Davoust B, et al. Prevalence of asymptomatic Tropheryma whipplei carriage among humans and nonhuman primates. J Infect Dis. 2008 Mar 15;197(6):880-7. http://jid.oxfordjournals.org/content/197/6/880.full http://www.ncbi.nlm.nih.gov/pubmed/18419351?tool=bestpractice.com [6]Ehrbar HU, Bauerfeind P, Dutly F, et al. PCR-positive tests for Tropheryma whippelii in patients without Whipple's disease. Lancet. 1999 Jun 26;353(9171):2214. http://www.ncbi.nlm.nih.gov/pubmed/10392994?tool=bestpractice.com [7]Amsler L, Bauernfeind P, Nigg C, et al. Prevalence of Tropheryma whipplei DNA in patients with various gastrointestinal diseases and in healthy controls. Infection. 2003 Mar;31(2):81-5. http://www.ncbi.nlm.nih.gov/pubmed/12682812?tool=bestpractice.com [8]Maibach RC, Dutly F, Altwegg M. Detection of Tropheryma whipplei DNA in feces by PCR using a target capture method. J Clin Microbiol. 2002 Jul;40(7):2466-71. http://jcm.asm.org/cgi/content/full/40/7/2466 http://www.ncbi.nlm.nih.gov/pubmed/12089263?tool=bestpractice.com [9]Zinkernagel AS, Gmur R, Fenner L, et al. Marginal and subgingival plaque: a natural habitat of Tropheryma whipplei? Infection. 2003 Mar;31(2):86-91. http://www.ncbi.nlm.nih.gov/pubmed/12682813?tool=bestpractice.com [10]Rolain JM, Fenollar F, Raoult D. False positive PCR detection of Tropheryma whipplei in the saliva of healthy people. BMC Microbiol. 2007 May 29;7:48. http://www.biomedcentral.com/1471-2180/7/48 http://www.ncbi.nlm.nih.gov/pubmed/17535423?tool=bestpractice.com ​​ A genetic predisposition for Whipple's disease seems to enable the establishment of an infection.[11]Schneider T, Moos V, Loddenkemper C, et al. Whipple's disease: new aspects of pathogenesis and treatment. Lancet Infect Dis. 2008 Mar;8(3):179-90. http://www.ncbi.nlm.nih.gov/pubmed/18291339?tool=bestpractice.com [12]Fenollar F, Puechal X, Raoult D. Whipple's disease. N Engl J Med. 2007 Jan 4;356(1):55-66.​​ Patients are typically middle-aged white men, who present with arthralgia, weight loss, diarrhoea, and fever.[11]Schneider T, Moos V, Loddenkemper C, et al. Whipple's disease: new aspects of pathogenesis and treatment. Lancet Infect Dis. 2008 Mar;8(3):179-90. http://www.ncbi.nlm.nih.gov/pubmed/18291339?tool=bestpractice.com [12]Fenollar F, Puechal X, Raoult D. Whipple's disease. N Engl J Med. 2007 Jan 4;356(1):55-66.[13]Dobbins WO 3rd. Is there an immune deficit in Whipple's disease? Dig Dis Sci. 1981 Mar;26(3):247-52. http://www.ncbi.nlm.nih.gov/pubmed/6165533?tool=bestpractice.com [14]Dobbins WO. Whipple's disease. Springfield, IL: Thomas; 1987.​​​​​​