MCID: WHP001
MIFTS: 53

Whipple Disease malady

Neuronal diseases, Gastrointestinal diseases, Infectious diseases categories

Summaries for Whipple Disease

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42NIH Rare Diseases, 43NINDS, 32MalaCards
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NIH Rare Diseases:42 Whipple disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats. the disorder usually occurs in the gastrointestinal system, but may affect any part of the body including the heart, lungs, brain, joints, and eyes. in the gastrointestinal system it interferes with the body's ability to absorb certain nutrients. this leads to a condition known as malabsorption. whipple disease causes weight loss, incomplete breakdown of carbohydrates or fats, and malfunctions of the immune system. it is caused by infection from bacteria called tropheryma whippelii. when recognized and treated, whipple disease can usually be cured. untreated, the disease may be fatal. last updated: 8/1/2012

MalaCards: Whipple Disease, also known as intestinal lipodystrophy, is related to arthritis and uveitis, and has symptoms including hypothyroidy, insulin resistance and galactorrhea. An important gene associated with Whipple Disease is IL16 (interleukin 16), and among its related pathways are Immune response Antigen presentation by MHC class II and Tuberculosis. The compounds nickel and ecori have been mentioned in the context of this disorder. Affiliated tissues include eye, lung and brain.

NINDS:43 Whipple's disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats. The disorder can affect any system in the body, including the central nervous system, but usually occurs in the gastrointestinal system. Gastrointestinal symptoms may include diarrhea, weight loss, fatigue, weakness, and abdominal bleeding and pain. Neurological symptoms may include abnormalities of eye and facial muscle movements, confusion, seizures, ataxia, memory loss, and vision impairment. Fever, cough, and joint soreness may also be present.

Aliases & Classifications for Whipple Disease

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 48Orphanet, 60UMLS, 39NCIt, 27ICD9CM, 56SNOMED-CT, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

48
intestinal lipodystrophy:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

whipple disease 8 42 48 60
intestinal lipodystrophy 8 42 43 48
intestinal lipophagic granulomatosis 42 48 60
secondary non-tropical sprue 42 48
whipple's disease 8 43
tropheryma whippelii infection 42


External Ids:

Disease Ontology8 DOID:8476
NCIt39 C85228
ICD9CM27 040.2
MeSH34 D008061
SNOMED-CT56 41545003
MESH via Orphanet35 C531849, D008061
ICD10 via Orphanet26 K90.8+, M14.8*
SNOMED-CT via Orphanet57 41545003
UMLS via Orphanet61 C0023788, C2930851

Related Diseases for Whipple Disease

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Whipple Disease:



Diseases related to whipple disease

Clinical Features for Whipple Disease

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48Orphanet
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Symptoms:

48 (show all 45)
  • hypothyroidy
  • insulin resistance
  • galactorrhea
  • hydrocephaly
  • cranial nerves palsy
  • peripheral neuropathy
  • cranial hypertension
  • encephalitis
  • ataxia/incoordination/trouble of the equilibrium
  • pyramidal syndrome
  • seizures/epilepsy/absences/spasms/status epilepticus
  • muscle weakness/flaccidity
  • myositis
  • anaemia
  • hyponatremia
  • thirst
  • humour troubles/anxiety/depression/apathy/euphoria/irritability
  • impotence/painful erection/priapism/erection troubles
  • edema of the legs/lower limbs
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • asthenia/fatigue/weakness
  • fever/chilling
  • wasted (excluding lipodystrophy)/poorly muscled build/cachexy
  • arthritis/synovitis/synovial proliferation
  • articular/joint pain/arthralgia
  • anorexia
  • psychic/psychomotor regression/dementia/intellectual decline
  • myoclonus/fasciculations
  • mediastinal/hilar adenopathies
  • malabsorption/chronic diarrhea/steatorrhea
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • myocardium anomalies/myocarditis
  • cough
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • thoracic/chest pain
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • diffuse/generalised skin hyperpigmentation/melanoderma
  • proptosis/exophthalmos
  • myalgia/muscular pain
  • sleep and vigilance disorders
  • hypotension
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • acute abdominal pain/colic

Drugs & Therapeutics for Whipple Disease

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Whipple Disease

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Anatomical Context for Whipple Disease

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Sources:
32MalaCards
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MalaCards organs/tissues related to Whipple Disease:

32
Eye, Lung, Brain, Heart, Skin, Liver, Spinal cord, Bone, B cells

Animal Models for Whipple Disease or affiliated genes

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Publications for Whipple Disease

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50PubMed
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Articles related to Whipple Disease:

(show top 50)    (show all 90)
idTitleAuthorsYear
1
Monoclonal B-cell lymphocyte proliferation in a patient presenting with historical Whipple disease. (23441662)
2013
2
Oculomasticatory myorhythmia and agrypnia excitata guide the diagnosis of Whipple disease. (24210608)
2013
3
Central nervous system involvement in Whipple disease: clinical study of 18 patients and long-term follow-up. (24145700)
2013
4
Whipple disease revealed by lung involvement: a case report and literature review. (22670021)
2012
5
Whipple disease mimicking adult-onset Still's disease and treated by anakinra: diagnosis using PCR. (22639877)
2012
6
Whipple disease a century after the initial description: increased recognition of unusual presentations, autoimmune comorbidities, and therapy effects. (22743287)
2012
7
Peripheral neuropathy in Whipples disease: a case report. (22716061)
2012
8
Isolated CNS Whipple disease with normal brain MRI and false-positive CSF 14-3-3 protein: a case report and review of the literature. (23170246)
2012
9
Whipple disease research accelerates. (21628651)
2011
10
The immune reconstitution inflammatory syndrome in whipple disease: a cohort study. (21135294)
2010
11
Whipple disease. (20224490)
2010
12
Whipple disease of the central nervous system: an unusual occurrence in association with acquired immune deficiency syndrome. (19612977)
2010
13
Infection: How should classic Whipple disease be managed? (20442732)
2010
14
Postoperative panophthalmitis caused by Whipple disease. (19402983)
2009
15
Isolated Whipple disease of the brain resembling a tumour. (19194653)
2009
16
Teaching NeuroImage: Oculomasticatory myorhythmia: pathognomonic phenomenology of Whipple disease. (18250283)
2008
17
Exacerbation of Whipple disease by immunosuppressants. (18496383)
2008
18
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. (18662136)
2008
19
Recurrent orbital inflammation and Whipple disease. (18379941)
2008
20
Isolated CNS Whipple disease: acute onset and relapsing-remitting course. (17577831)
2007
21
Whipple disease revealed by musculocutaneous symptoms, with muscle biopsy cultures positive for Tropheryma whipplei. (17900961)
2007
22
A patient with cerebral Whipple disease with gastric involvement but no gastrointestinal symptoms: a consequence of local protective immunity? (17371903)
2007
23
A case of Whipple disease with pleural effusion diagnosed by means of PCR. (17181935)
2006
24
Whipple disease: intestinal infiltrating cells exhibit a transcriptional pattern of M2/alternatively activated macrophages. (16206080)
2005
25
Neurological manifestations of Whipple disease. (14978404)
2004
26
Whipple disease associated with giardiasis. (12964113)
2003
27
Whipple disease. (14565900)
2003
28
Intracerebral Whipple disease: unusual location and bone destruction. Case report. (12405392)
2002
29
Spinal cord involvement in CNS Whipple disease: an ongoing experience in the MR imaging era. (11827892)
2002
30
Primary diagnosis of whipple disease manifesting as lymphadenopathy: use of polymerase chain reaction for detection of Tropheryma whippelii. (11764080)
2001
31
Tropheryma whippelii DNA is rare in the intestinal mucosa of patients without other evidence of Whipple disease. (11177314)
2001
32
Culture and immunological detection of Tropheryma whippelii from the duodenum of a patient with Whipple disease. (11209175)
2001
33
Successful treatment of Whipple disease diagnosed 36 years after symptom onset. (11605693)
2001
34
Spinal cord involvement in CNS Whipple disease: an ongoing experience in the magnetic resonance imaging era. (11733340)
2001
35
Diffuse lesions in the CNS revealed by MR imaging in a case of Whipple disease. (11237971)
2001
36
Granulomatous nephritis as the first manifestation of Whipple disease. (10627265)
2000
37
Cognitive and behavioral abnormalities in a case of central nervous system Whipple disease. (10714668)
2000
38
Ocular manifestations of Whipple disease: an atypical presentation. (9747687)
1998
39
Whipple disease presenting as posterior uveitis without prominent gastrointestinal symptoms. (9683160)
1998
40
Aortic valve endocarditis with Whipple disease. (9163306)
1997
41
Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. SociActAc Nationale FranAsaise de MAcdecine Interne. (9193452)
1997
42
Diagnosis and monitoring of Whipple disease by polymerase chain reaction. (9092317)
1997
43
Whipple disease of the mediastinum. (1692443)
1990
44
MR imaging of CNS relapse of Whipple disease. (1697867)
1990
45
The evolving natural history of neurologic involvement in Whipple disease: a hypothesis. (2456052)
1988
46
Whipple disease. (2421412)
1986
47
Mycobacterium avium-intracellulare complex enteritis: pseudo-Whipple disease in AIDS. (2580424)
1985
48
Computed tomography in Whipple disease. (6195202)
1983
49
Letter: Whipple disease. (58083)
1976
50
Whipple disease. Occurrence in a 76-year-old man with a 20-year prodrome of arthritis. (48564)
1975

Genetic Variations for Whipple Disease

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Expression for genes affiliated with Whipple Disease

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Whipple Disease

Search GEO for disease gene expression data for Whipple Disease.

Pathways for genes affiliated with Whipple Disease

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12EMD Millipore, 29KEGG, 51QIAGEN, 53Reactome
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Compounds for genes affiliated with Whipple Disease

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44Novoseek, 24HMDB
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Compounds related to Whipple Disease according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1nickel44 2410.7HLA-DQB1, HSPD1
2ecori449.4HLA-B, HLA-DQB1
3rantes449.1IL16, HLA-B, HSPD1
4ribonucleic acid448.8HSPD1, HLA-B, PRL

GO Terms for genes affiliated with Whipple Disease

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16Gene Ontology
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Cellular components related to Whipple Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1MHC class II protein complexGO:0426139.5HLA-DRB1, HLA-DQB1
2trans-Golgi network membraneGO:0325889.5HLA-DQB1, HLA-DRB1
3clathrin-coated endocytic vesicle membraneGO:0306699.4HLA-DRB1, HLA-DQB1
4transport vesicle membraneGO:0306589.2HLA-DQB1, HLA-DRB1
5integral to lumenal side of endoplasmic reticulum membraneGO:0715569.0HLA-DQB1, HLA-B, HLA-DRB1
6ER to Golgi transport vesicle membraneGO:0125079.0HLA-DRB1, HLA-B, HLA-DQB1

Biological processes related to Whipple Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1immunoglobulin production involved in immunoglobulin mediated immune responseGO:0023819.6HLA-DRB1, HLA-DQB1
2humoral immune response mediated by circulating immunoglobulinGO:0024559.4HLA-DQB1, HLA-DRB1
3interferon-gamma-mediated signaling pathwayGO:0603339.0HLA-DQB1, HLA-B, HLA-DRB1
4detection of bacteriumGO:0160459.0HLA-DRB1, HLA-B
5cytokine-mediated signaling pathwayGO:0192218.9HLA-B, HLA-DQB1, HLA-DRB1
6immune responseGO:0069558.6IL16, HLA-DRB1, HLA-B, HLA-DQB1

Molecular functions related to Whipple Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1peptide antigen bindingGO:0426059.3HLA-DRB1, HLA-B
2MHC class II receptor activityGO:0323959.2HLA-DRB1, HLA-DQB1

Products for genes affiliated with Whipple Disease

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  • Antibodies
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  • Lysates
  • Antibodies

Sources for Whipple Disease

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet