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Whipple disease

MedGen UID:
7347
•Concept ID:
C0023788
•
Disease or Syndrome
Synonyms: Intestinal lipodystrophy; Intestinal lipophagic granulomatosis; Secondary Non-tropical Sprue; Tropheryma whippelii infection; Whipple's disease
SNOMED CT: WD - Whipple's disease (41545003); Intestinal Whipple's disease (41545003); Whipple disease (41545003); Whipple's disease (41545003); Intestinal lipodystrophy (41545003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0005116
Orphanet: ORPHA3452

Definition

A rare chronic infectious disorder in which almost all organ systems can be invaded by the rod-shaped bacterium Tropheryma whipplei (<i>TW</i>). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWhipple disease

Professional guidelines

PubMed

Author response: IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease.
Morales-Briceño H, Fung V
Neurology 2019 Mar 12;92(11):544-545. doi: 10.1212/WNL.0000000000007088. PMID: 30858248
Reader response: IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease.
Bally JF, Lang AE
Neurology 2019 Mar 12;92(11):544. doi: 10.1212/WNL.0000000000007087. PMID: 30858247
Value of Tropheryma whipplei quantitative polymerase chain reaction assay for the diagnosis of Whipple disease: usefulness of saliva and stool specimens for first-line screening.
Fenollar F, Laouira S, Lepidi H, Rolain JM, Raoult D
Clin Infect Dis 2008 Sep 1;47(5):659-67. doi: 10.1086/590559. PMID: 18662136

Recent clinical studies

Etiology

Impaired intracellular pathogen clearance and inflammatory joint disease: Is Whipple's disease a guiding light?
Berthelot JM, Puéchal X
Joint Bone Spine 2018 Oct;85(5):531-536. Epub 2017 Sep 28 doi: 10.1016/j.jbspin.2017.09.007. PMID: 28965939
Whipple's arthritis.
Puéchal X
Joint Bone Spine 2016 Dec;83(6):631-635. Epub 2016 Aug 5 doi: 10.1016/j.jbspin.2016.07.001. PMID: 27502365
Infective endocarditis.
Moreillon P, Que YA
Lancet 2004 Jan 10;363(9403):139-49. doi: 10.1016/S0140-6736(03)15266-X. PMID: 14726169
Whipple's disease.
Marth T, Raoult D
Lancet 2003 Jan 18;361(9353):239-46. doi: 10.1016/S0140-6736(03)12274-X. PMID: 12547551
Whipple's disease.
Ratnaike RN
Postgrad Med J 2000 Dec;76(902):760-6. doi: 10.1136/pmj.76.902.760. PMID: 11085766Free PMC Article

Diagnosis

Whipple's disease and Tropheryma whipplei infections: from bench to bedside.
Boumaza A, Ben Azzouz E, Arrindell J, Lepidi H, Mezouar S, Desnues B
Lancet Infect Dis 2022 Oct;22(10):e280-e291. Epub 2022 Apr 12 doi: 10.1016/S1473-3099(22)00128-1. PMID: 35427488
Whipple's disease and Tropheryma whipplei infections: when to suspect them and how to diagnose and treat them.
Lagier JC, Raoult D
Curr Opin Infect Dis 2018 Dec;31(6):463-470. doi: 10.1097/QCO.0000000000000489. PMID: 30299363
Whipple's disease.
El-Abassi R, Soliman MY, Williams F, England JD
J Neurol Sci 2017 Jun 15;377:197-206. Epub 2017 Feb 16 doi: 10.1016/j.jns.2017.01.048. PMID: 28477696
Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections.
Dolmans RA, Boel CH, Lacle MM, Kusters JG
Clin Microbiol Rev 2017 Apr;30(2):529-555. doi: 10.1128/CMR.00033-16. PMID: 28298472Free PMC Article
Tropheryma whipplei infection and Whipple's disease.
Marth T, Moos V, Müller C, Biagi F, Schneider T
Lancet Infect Dis 2016 Mar;16(3):e13-22. Epub 2016 Feb 6 doi: 10.1016/S1473-3099(15)00537-X. PMID: 26856775

Therapy

Whipple's disease and Tropheryma whipplei infections: when to suspect them and how to diagnose and treat them.
Lagier JC, Raoult D
Curr Opin Infect Dis 2018 Dec;31(6):463-470. doi: 10.1097/QCO.0000000000000489. PMID: 30299363
Whipple's disease.
Marth T
Acta Clin Belg 2016 Dec;71(6):373-378. Epub 2016 Nov 24 doi: 10.1080/17843286.2016.1256586. PMID: 27884091
Tropheryma whipplei infection and Whipple's disease.
Marth T, Moos V, Müller C, Biagi F, Schneider T
Lancet Infect Dis 2016 Mar;16(3):e13-22. Epub 2016 Feb 6 doi: 10.1016/S1473-3099(15)00537-X. PMID: 26856775
Whipple's disease.
Schwartzman S, Schwartzman M
Rheum Dis Clin North Am 2013 May;39(2):313-21. doi: 10.1016/j.rdc.2013.03.005. PMID: 23597966
Whipple's disease.
Biagi F, Trotta L, Corazza GR
Intern Emerg Med 2012 Oct;7 Suppl 3:S209-13. doi: 10.1007/s11739-012-0801-1. PMID: 23073859

Prognosis

Whipple's disease and Tropheryma whipplei infections: when to suspect them and how to diagnose and treat them.
Lagier JC, Raoult D
Curr Opin Infect Dis 2018 Dec;31(6):463-470. doi: 10.1097/QCO.0000000000000489. PMID: 30299363
Whipple's disease.
El-Abassi R, Soliman MY, Williams F, England JD
J Neurol Sci 2017 Jun 15;377:197-206. Epub 2017 Feb 16 doi: 10.1016/j.jns.2017.01.048. PMID: 28477696
Whipple's arthritis.
Puéchal X
Joint Bone Spine 2016 Dec;83(6):631-635. Epub 2016 Aug 5 doi: 10.1016/j.jbspin.2016.07.001. PMID: 27502365
Tropheryma whipplei infection and Whipple's disease.
Marth T, Moos V, Müller C, Biagi F, Schneider T
Lancet Infect Dis 2016 Mar;16(3):e13-22. Epub 2016 Feb 6 doi: 10.1016/S1473-3099(15)00537-X. PMID: 26856775
Whipple's disease.
Fantry GT, James SP
Dig Dis 1995 Mar-Apr;13(2):108-18. doi: 10.1159/000171492. PMID: 7586631

Clinical prediction guides

Whipple's disease and Tropheryma whipplei infections: when to suspect them and how to diagnose and treat them.
Lagier JC, Raoult D
Curr Opin Infect Dis 2018 Dec;31(6):463-470. doi: 10.1097/QCO.0000000000000489. PMID: 30299363
Tropheryma whipplei endocarditis.
Fenollar F, Célard M, Lagier JC, Lepidi H, Fournier PE, Raoult D
Emerg Infect Dis 2013 Nov;19(11):1721-30. doi: 10.3201/eid1911.121356. PMID: 24207100Free PMC Article
Whipple's disease: a rare disease revisited.
Afshar P, Redfield DC, Higginbottom PA
Curr Gastroenterol Rep 2010 Aug;12(4):263-9. doi: 10.1007/s11894-010-0110-7. PMID: 20532708
Progress, problems, and perspectives in diagnosis and treatment of Whipple's disease.
Mahnel R, Marth T
Clin Exp Med 2004 Sep;4(1):39-43. doi: 10.1007/s10238-004-0036-1. PMID: 15598084
Whipple's disease.
Fenollar F, Raoult D
Curr Gastroenterol Rep 2003 Oct;5(5):379-85. doi: 10.1007/s11894-003-0050-6. PMID: 12959718

Recent systematic reviews

Whipple's disease-associated infective endocarditis: a systematic review.
Ioannou P, Kourtidis M, Mytilinis DO, Psyllaki A, Baliou S, Kofteridis D
Infect Dis (Lond) 2023 Jul;55(7):447-457. Epub 2023 May 17 doi: 10.1080/23744235.2023.2214610. PMID: 37198913
Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple's disease.
Manini A, Querzola G, Lovati C, Pantoni L
Neurol Sci 2022 Feb;43(2):907-926. Epub 2022 Jan 3 doi: 10.1007/s10072-021-05844-5. PMID: 34981284Free PMC Article
Systematic review of movement disorders and oculomotor abnormalities in Whipple's disease.
Bally JF, Méneret A, Roze E, Anderson M, Grabli D, Lang AE
Mov Disord 2018 Nov;33(11):1700-1711. Epub 2018 Oct 19 doi: 10.1002/mds.27419. PMID: 30338868
Systematic review: Whipple's disease (Tropheryma whipplei infection) and its unmasking by tumour necrosis factor inhibitors.
Marth T
Aliment Pharmacol Ther 2015 Apr;41(8):709-24. Epub 2015 Feb 19 doi: 10.1111/apt.13140. PMID: 25693648
Isolated intracranial Whipple's disease--report of a rare case and review of the literature.
Mohamed W, Neil E, Kupsky WJ, Juhász C, Mittal S, Santhakumar S
J Neurol Sci 2011 Sep 15;308(1-2):1-8. Epub 2011 Jun 22 doi: 10.1016/j.jns.2011.05.029. PMID: 21696776

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