MCID: WHP001
MIFTS: 47

Whipple Disease malady

Neuronal, Gastrointestinal, Infectious categories

Summaries for Whipple Disease

Sources:
43NIH Rare Diseases, 44NINDS, 33MalaCards
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NIH Rare Diseases:43 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 acute abdominal pain/colic, malabsorption/chronic diarrhea/steatorrhea and mediastinal/hilar adenopathies. 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 brain, spinal cord and heart.

NINDS:44 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

Sources:
8Disease Ontology, 43NIH Rare Diseases, 44NINDS, 49Orphanet, 61UMLS, 40NCIt, 27ICD9CM, 57SNOMED-CT, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Infectious
Anatomical: Neuronal, Gastrointestinal


Characteristics (Orphanet epidemiological data):

49
intestinal lipodystrophy:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

whipple disease 8 43 49 61
intestinal lipodystrophy 8 43 44 49
intestinal lipophagic granulomatosis 43 49 61
secondary non-tropical sprue 43 49
whipple's disease 8 44
tropheryma whippelii infection 43


External Ids:

Disease Ontology8 DOID:8476
NCIt40 C85228
ICD9CM27 040.2
MeSH35 D008061
SNOMED-CT57 41545003
MESH via Orphanet36 C531849, D008061
ICD10 via Orphanet26 K90.8+, M14.8*
SNOMED-CT via Orphanet58 41545003
UMLS via Orphanet62 C0023788, C2930851

Related Diseases for Whipple Disease

Sources:
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

Sources:
49Orphanet
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Symptoms:

49 (show all 45)
  • acute abdominal pain/colic
  • malabsorption/chronic diarrhea/steatorrhea
  • mediastinal/hilar adenopathies
  • myoclonus/fasciculations
  • psychic/psychomotor regression/dementia/intellectual decline
  • anorexia
  • humour troubles/anxiety/depression/apathy/euphoria/irritability
  • articular/joint pain/arthralgia
  • arthritis/synovitis/synovial proliferation
  • wasted (excluding lipodystrophy)/poorly muscled build/cachexy
  • fever/chilling
  • asthenia/fatigue/weakness
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • hypotension
  • sleep and vigilance disorders
  • myalgia/muscular pain
  • proptosis/exophthalmos
  • diffuse/generalised skin hyperpigmentation/melanoderma
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • thoracic/chest pain
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • cough
  • myocardium anomalies/myocarditis
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • edema of the legs/lower limbs
  • impotence/painful erection/priapism/erection troubles
  • 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

Drugs & Therapeutics for Whipple Disease

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Whipple Disease

Drug clinical trials:

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Search NIH Clinical Center for Whipple Disease

Search CenterWatch for Whipple Disease

Genetic Tests for Whipple Disease

Anatomical Context for Whipple Disease

Sources:
33MalaCards
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MalaCards organs/tissues related to Whipple Disease:

33
Brain, Spinal cord, Heart, Skeletal muscle, Colon, Liver, Lung, B cells

Animal Models for Whipple Disease or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Whipple Disease

Sources:
51PubMed
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Articles related to Whipple Disease:

(show top 50)    (show all 132)
idTitleAuthorsYear
1
A severe Whipple disease with an immune reconstitution inflammatory syndrome: An additional case of thalidomide efficiency. (24289960)
2013
2
Whipple disease diagnosed with PCR using formalin-fixed paraffin-embedded specimens of the intestinal mucosa. (23318851)
2013
3
Palindromic rheumatism: consider Whipple's disease. (23992271)
2013
4
Oculomasticatory myorhythmia and agrypnia excitata guide the diagnosis of Whipple disease. (24210608)
2013
5
Central nervous system involvement in Whipple disease: clinical study of 18 patients and long-term follow-up. (24145700)
2013
6
Whipple's disease presenting with segmental myoclonus and hypersomnia. (22976777)
2012
7
Whipple disease a century after the initial description: increased recognition of unusual presentations, autoimmune comorbidities, and therapy effects. (22743287)
2012
8
Peripheral neuropathy in Whipples disease: a case report. (22716061)
2012
9
Connecting the dots: the many systemic manifestations of whipple disease. (22347837)
2012
10
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
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
Scurvy as the presenting illness of Whipple's disease exacerbated by treatment with etanercept in a patient with ankylosing spondylitis. (20439534)
2010
15
The HLA alleles DRB1*13 and DQB1*06 are associated to Whipple's disease. (19208355)
2009
16
A case of Noonan syndrome and Whipple's disease in the same patient. (19322929)
2009
17
Reversible pulmonary hypertension in Whipple disease: a case report with clinicopathological implications, and literature review. (21686934)
2009
18
Whipple disease. (18301263)
2008
19
Whipple's procedure in a renal transplant recipient with polycystic liver disease. (18648145)
2008
20
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
21
Isolated CNS Whipple disease with a variant of oculofacial-skeletal myorhythmia (OFSM). (17846404)
2007
22
Premature ovarian failure in suspected Whipple's disease. (18078849)
2007
23
A patient with cerebral Whipple disease with gastric involvement but no gastrointestinal symptoms: a consequence of local protective immunity? (17371903)
2007
24
Intestinal histoplasmosis mimicking Whipple's disease. (16430478)
2006
25
Decreased levels of interleukin-12p40 in the serum of patients with Whipple's disease. (15875203)
2006
26
IL-16 is critical for Tropheryma whipplei replication in Whipple's disease. (16177102)
2005
27
Whipple disease of the central nervous system. (12964109)
2003
28
Neurologic presentation of Whipple disease: report of 12 cases and review of the literature. (12441901)
2002
29
Primary diagnosis of whipple disease manifesting as lymphadenopathy: use of polymerase chain reaction for detection of Tropheryma whippelii. (11764080)
2001
30
Culture and immunological detection of Tropheryma whippelii from the duodenum of a patient with Whipple disease. (11209175)
2001
31
Successful treatment of Whipple disease diagnosed 36 years after symptom onset. (11605693)
2001
32
Rhythmic tremor of the palate and other cranial limb muscles, with cerebellar ataxia: consider Whipple's disease. (11481720)
2001
33
Spinal cord involvement in CNS Whipple disease: an ongoing experience in the magnetic resonance imaging era. (11733340)
2001
34
Antibodies against recombinant heat shock protein 65 of Tropheryma whipplei in patients with and without Whipple's disease. (11714520)
2001
35
Whipple's disease mimicking linitis plastica. (10223387)
1999
36
Diagnostic utility of the polymerase chain reaction in 2 cases of suspected Whipple disease. (9554687)
1998
37
Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. SociActAc Nationale FranAsaise de MAcdecine Interne. (9193452)
1997
38
Diagnosis and monitoring of Whipple disease by polymerase chain reaction. (9092317)
1997
39
Detection of a clonal BCL2 gene rearrangement in tissues from a patient with Whipple disease. (8633834)
1996
40
Enteropathic arthritis, Whipple's disease, juvenile spondyloarthropathy, uveitis, and SAPHO syndrome. (7547105)
1995
41
Permanent bruxism as a manifestation of the oculo-facial syndrome related to systemic Whipple's disease. (1372960)
1992
42
Granulomatous gastritis and Whipple's disease. (1372792)
1992
43
MR imaging of CNS relapse of Whipple disease. (1697867)
1990
44
Oculo-facial-skeletal myorhythmia in Whipple disease: treatment with ceftriaxone. (1689977)
1990
45
Mycobacterium avium-intracellulare complex enteritis: pseudo-Whipple disease in AIDS. (2580424)
1985
46
Scurvy as an initial manifestation of Whipple's disease. (6203453)
1984
47
Computed tomography in Whipple disease. (6195202)
1983
48
Whipple disease: light and electron microscopy study. (4138040)
1974
49
Canine histiocytic ulcerative colitis. A condition resembling Whipple's disease, colonic histiocytosis, and malakoplakia in man. (4169328)
1968
50
Idiopathic steatorrhea; with report of a case of Whipple's disease. (18882940)
1948

Genetic Variations for Whipple Disease

Expression for genes affiliated with Whipple Disease

Sources:
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

Sources:
12EMD Millipore, 30KEGG, 52QIAGEN, 54Reactome
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Compounds for genes affiliated with Whipple Disease

Sources:
45Novoseek, 24HMDB
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Compounds related to Whipple Disease according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1nickel45 2410.7HLA-DQB1, HSPD1
2ecori459.4HLA-DQB1, HLA-B
3rantes459.1HSPD1, HLA-B, IL16
4ribonucleic acid458.8HSPD1, HLA-B, PRL

GO Terms for genes affiliated with Whipple Disease

Sources:
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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Whipple Disease

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet