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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

Diagnosis and treatment of Tropheryma whipplei infection in patients with inflammatory rheumatic disease: Data from the French Tw-IRD registry.
Caillet Portillo D, Puéchal X, Masson M, Kostine M, Michaut A, Ramon A, Wendling D, Costedoat-Chalumeau N, Richette P, Marotte H, Vix-Portet J, Dubost JJ, Ottaviani S, Mouterde G, Grasland A, Frazier A, Germain V, Coury F, Tournadre A, Soubrier M, Cavalie L, Brevet P, Zabraniecki L, Jamard B, Couture G, Arnaud L, Richez C, Degboé Y, Ruyssen-Witrand A, Constantin A
J Infect 2024 Feb;88(2):132-138. Epub 2023 Dec 22 doi: 10.1016/j.jinf.2023.12.010. PMID: 38141787
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

Recent clinical studies

Etiology

Histochemistry in the diagnosis of non-neoplastic gastrointestinal disorders.
Fitzgibbons PL
Semin Diagn Pathol 2018 Nov;35(6):370-380. Epub 2018 Oct 16 doi: 10.1053/j.semdp.2018.10.002. PMID: 30366790
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.
Valkov H, Kovacheva-Slavova M, Lyutakov I, Vladimirov B, Penchev P
Br J Hosp Med (Lond) 2021 Mar 2;82(3):1-2. Epub 2021 Mar 31 doi: 10.12968/hmed.2020.0614. PMID: 33792387
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.
Marth T
Acta Clin Belg 2016 Dec;71(6):373-378. Epub 2016 Nov 24 doi: 10.1080/17843286.2016.1256586. PMID: 27884091
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
Whipple's disease.
Tsarfati EM, Sutherland R
Br J Hosp Med (Lond) 2016 Jun;77(6):C82-5. doi: 10.12968/hmed.2016.77.6.C82. PMID: 27269764
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.
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

Infectious leukoencephalopathies.
Jakabek D, Chaganti J, Brew BJ
Handb Clin Neurol 2024;204:431-453. doi: 10.1016/B978-0-323-99209-1.00016-8. PMID: 39322393
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.
Fenollar F, Puéchal X, Raoult D
N Engl J Med 2007 Jan 4;356(1):55-66. doi: 10.1056/NEJMra062477. PMID: 17202456

Clinical prediction guides

Whipple's disease: diagnosis and predictive factors of relapse.
Ferrieres L, Laroche M, Bonnet D, Alric L
Eur J Gastroenterol Hepatol 2020 Mar;32(3):325-328. doi: 10.1097/MEG.0000000000001611. PMID: 31764405
Histochemistry in the diagnosis of non-neoplastic gastrointestinal disorders.
Fitzgibbons PL
Semin Diagn Pathol 2018 Nov;35(6):370-380. Epub 2018 Oct 16 doi: 10.1053/j.semdp.2018.10.002. PMID: 30366790
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.
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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