MCID: WHP001
MIFTS: 49

Whipple Disease

Categories: Rare diseases, Neuronal diseases, Gastrointestinal diseases, Infectious diseases

Aliases & Classifications for Whipple Disease

MalaCards integrated aliases for Whipple Disease:

Name: Whipple Disease 12 76 53 59 44 15 73
Intestinal Lipodystrophy 12 76 53 54 59
Intestinal Lipophagic Granulomatosis 53 59 73
Secondary Non-Tropical Sprue 53 59
Whipple's Disease 12 54
Tropheryma Whippelii Infection 53
Whipples Disease 55

Characteristics:

Orphanet epidemiological data:

59
whipple disease
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe),1-9/1000000 (Italy); Age of onset: All ages;

Classifications:



Summaries for Whipple Disease

NINDS : 54 Whipple's disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats. Caused by the bacterium Tropheryma whipplei, the disorder can affect any system in the body, including the brain, eyes, heart, joints, and lungs, but usually occurs in the gastrointestinal system. Neurological symptoms occur in up to 40 percent of individuals and may include dementia, abnormalities of eye and facial muscle movements, headaches, seizures, loss of muscle control, memory loss, weakness, and vision problems. Gastrointestinal symptoms may include diarrhea, weight loss, fatigue, weakness, and abdominal bleeding and pain. Fever, cough, anemia, heart and lung damage, darkening of the skin, and joint soreness may also be present. The disease is more common in men and neurological symptoms are more common in individuals who have severe abdominal disease. Rarely, neurological symptoms may appear without gastrointestinal symptoms and can mimic symptoms of almost any neurologic disease.

MalaCards based summary : Whipple Disease, also known as intestinal lipodystrophy, is related to hypersomnia and panuveitis. An important gene associated with Whipple Disease is IL16 (Interleukin 16), and among its related pathways/superpathways are Epstein-Barr virus infection and Tuberculosis. Affiliated tissues include brain, lung and eye, and related phenotypes are hydrocephalus and proptosis

NIH Rare Diseases : 53 Whipple disease is an infectious bacterial disease that affects many different organ systems and interferes with the body's ability to process (metabolize) fats. The disease 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 problems with the immune system. It is caused by infection from bacteria called Tropheryma whipplei. When recognized and treated, Whipple disease can usually be cured. Untreated, the disease may be fatal.

Wikipedia : 76 Whipple\'s disease is a rare, systemic infectious disease caused by the bacterium Tropheryma whipplei.... more...

Related Diseases for Whipple Disease

Diseases related to Whipple Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 59)
# Related Disease Score Top Affiliating Genes
1 hypersomnia 30.0 HLA-DQB1 HLA-DRB1
2 panuveitis 29.0 HLA-DQB1 HLA-DRB1 MRAP
3 actinic prurigo 10.4 CD58 HLA-DRB1
4 limbic encephalitis with lgi1 antibodies 10.4 HLA-DQB1 HLA-DRB1
5 metal allergy 10.4 HLA-DQB1 HLA-DRB1
6 focal epithelial hyperplasia 10.4 HLA-DQB1 HLA-DRB1
7 lichen planopilaris 10.4 HLA-DQB1 HLA-DRB1
8 type ii mixed cryoglobulinemia 10.4 HLA-DQB1 HLA-DRB1
9 beryllium disease 10.4 HLA-DQB1 HLA-DRB1
10 recurrent respiratory papillomatosis 10.4 HLA-DQB1 HLA-DRB1
11 autoimmune pancreatitis 10.4 HLA-DQB1 HLA-DRB1
12 chronic beryllium disease 10.4 HLA-DQB1 HLA-DRB1
13 pityriasis rosea 10.4 HLA-DQB1 HLA-DRB1
14 lichen sclerosus 10.4 HLA-DQB1 HLA-DRB1
15 acute disseminated encephalomyelitis 10.4 HLA-DQB1 HLA-DRB1
16 pediatric multiple sclerosis 10.3 HLA-DQB1 HLA-DRB1
17 osteonecrosis of the jaw 10.3 HLA-DQB1 HLA-DRB1
18 idiopathic bronchiectasis 10.3 HLA-DQB1 HLA-DRB1
19 peanut allergy 10.3 HLA-DQB1 HLA-DRB1
20 oligoarticular juvenile idiopathic arthritis 10.3 HLA-DQB1 HLA-DRB1
21 geographic tongue 10.3 HLA-DQB1 HLA-DRB1
22 severe hemophilia a 10.2 HLA-DQB1 HLA-DRB1
23 ocular cicatricial pemphigoid 10.2 HLA-DQB1 HLA-DRB1
24 paraneoplastic pemphigus 10.1 HLA-DQB1 HLA-DRB1
25 oral lichen planus 10.1 CD58 HSPD1
26 mixed connective tissue disease 10.1 HLA-DRB1 HSPD1
27 vogt-koyanagi-harada disease 10.1 HLA-DQB1 HLA-DRB1
28 pelvic inflammatory disease 10.1 HLA-DQB1 HSPD1
29 rubella 10.0 HLA-DQB1 HLA-DRB1
30 palindromic rheumatism 10.0 HLA-DRB1 MRAP
31 urethritis 10.0 HSPD1 MRAP
32 cervicitis 10.0 HSPD1 MRAP
33 mesenteric lymphadenitis 10.0 HSPD1 MRAP
34 graft-versus-host disease 9.9 HLA-DQB1 HLA-DRB1
35 pulmonary hypertension 9.9
36 arthritis 9.9
37 cerebritis 9.9
38 autoimmune hepatitis 9.8 HLA-DQB1 HLA-DRB1
39 bone inflammation disease 9.8 HLA-DRB1 MRAP
40 uveitis 9.8
41 diabetes mellitus, insulin-dependent 9.7 HLA-DQB1 HLA-DRB1 HSPD1
42 autoimmune disease 9.7 HLA-DQB1 HLA-DRB1 HSPD1
43 aceruloplasminemia 9.6
44 ataxia-oculomotor apraxia 3 9.6
45 keratopathy 9.6
46 thrombosis 9.6
47 endocarditis 9.6
48 giardiasis 9.6
49 posterior uveitis 9.6
50 dementia 9.6

Graphical network of the top 20 diseases related to Whipple Disease:



Diseases related to Whipple Disease

Symptoms & Phenotypes for Whipple Disease

Human phenotypes related to Whipple Disease:

59 32 (show all 47)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000238
2 proptosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000520
3 uveitis 59 32 frequent (33%) Frequent (79-30%) HP:0000554
4 depressivity 59 32 hallmark (90%) Very frequent (99-80%) HP:0000716
5 hypothyroidism 59 32 occasional (7.5%) Occasional (29-5%) HP:0000821
6 insulin resistance 59 32 occasional (7.5%) Occasional (29-5%) HP:0000855
7 seizures 59 32 occasional (7.5%) Occasional (29-5%) HP:0001250
8 ataxia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001251
9 muscle weakness 59 32 occasional (7.5%) Occasional (29-5%) HP:0001324
10 myoclonus 59 32 hallmark (90%) Very frequent (99-80%) HP:0001336
11 arthritis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001369
12 myocardial infarction 59 32 occasional (7.5%) Occasional (29-5%) HP:0001658
13 pericarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001701
14 splenomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0001744
15 anemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001903
16 fever 59 32 hallmark (90%) Very frequent (99-80%) HP:0001945
17 polydipsia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001959
18 diarrhea 59 32 hallmark (90%) Very frequent (99-80%) HP:0002014
19 malabsorption 59 32 hallmark (90%) Very frequent (99-80%) HP:0002024
20 abdominal pain 59 32 hallmark (90%) Very frequent (99-80%) HP:0002027
21 anorexia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002039
22 respiratory insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0002093
23 pleuritis 59 32 frequent (33%) Frequent (79-30%) HP:0002102
24 gastrointestinal hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0002239
25 hepatomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0002240
26 sleep disturbance 59 32 frequent (33%) Frequent (79-30%) HP:0002360
27 developmental regression 59 32 hallmark (90%) Very frequent (99-80%) HP:0002376
28 encephalitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0002383
29 increased intracranial pressure 59 32 occasional (7.5%) Occasional (29-5%) HP:0002516
30 hypotension 59 32 frequent (33%) Frequent (79-30%) HP:0002615
31 arthralgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002829
32 hyponatremia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002902
33 myalgia 59 32 frequent (33%) Frequent (79-30%) HP:0003326
34 cachexia 59 32 hallmark (90%) Very frequent (99-80%) HP:0004326
35 cranial nerve paralysis 59 32 occasional (7.5%) Occasional (29-5%) HP:0006824
36 abnormal pyramidal signs 59 32 occasional (7.5%) Occasional (29-5%) HP:0007256
37 generalized hyperpigmentation 59 32 occasional (7.5%) Occasional (29-5%) HP:0007440
38 peripheral neuropathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0009830
39 edema of the lower limbs 59 32 occasional (7.5%) Occasional (29-5%) HP:0010741
40 fatigue 59 32 hallmark (90%) Very frequent (99-80%) HP:0012378
41 cough 59 32 occasional (7.5%) Occasional (29-5%) HP:0012735
42 myocarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012819
43 myositis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100614
44 erectile abnormalities 59 32 occasional (7.5%) Occasional (29-5%) HP:0100639
45 mediastinal lymphadenopathy 59 32 hallmark (90%) Very frequent (99-80%) HP:0100721
46 chest pain 59 32 occasional (7.5%) Occasional (29-5%) HP:0100749
47 galactorrhea 59 32 occasional (7.5%) Occasional (29-5%) HP:0100829

Drugs & Therapeutics for Whipple Disease

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Detection and Characteristic of Whipple Diseases in the Great Britany Completed NCT03350685
2 Chronic Infection With Tropheryma Whipplei: Risk Factors Related to the Host Recruiting NCT02868450 Not Applicable

Search NIH Clinical Center for Whipple Disease

Cochrane evidence based reviews: whipple disease

Genetic Tests for Whipple Disease

Anatomical Context for Whipple Disease

MalaCards organs/tissues related to Whipple Disease:

41
Brain, Lung, Eye, Heart, Skin, Spinal Cord, B Cells

Publications for Whipple Disease

Articles related to Whipple Disease:

(show top 50) (show all 111)
# Title Authors Year
1
Whipple Disease Presenting as Cystic Brain Tumor: Case Report and Review of the Literature. ( 29707758 )
2018
2
Tropheryma whipplei Infection (Whipple Disease) in the USA. ( 29572616 )
2018
3
IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease. ( 29769372 )
2018
4
Cytology of cerebrospinal fluid in CNS Whipple disease. ( 28770535 )
2017
5
Genotypic analysis of Tropheryma whipplei from patients with Whipple disease in the Americas. ( 28385924 )
2017
6
Ultrastructural changes of small intestine in Whipple disease patient. ( 28230475 )
2017
7
A case of rapidly progressive dementia: Whipple disease of CNS. ( 29079886 )
2017
8
Whipple Disease ( 28722966 )
2017
9
Pseudo-Whipple Disease Cutaneous Lesions. ( 27097339 )
2016
10
Prosopagnosia as the Presenting Symptom of Whipple Disease. ( 27336807 )
2016
11
Deep Vein Thrombosis as Initial Manifestation of Whipple Disease. ( 27920655 )
2016
12
Whipple disease confined to the central nervous system presenting as a cystic tumor: Case report and review of literature. ( 27593833 )
2016
13
Primary Whipple disease of the brain: case report with long-term clinical and MRI follow-up. ( 26445540 )
2015
14
Gastrointestinal diagnosis of classical whipple disease: clinical, endoscopic, and histopathologic features in 191 patients. ( 25881849 )
2015
15
Atypical presentation of Whipple disease with no diarrhea. ( 26275278 )
2015
16
Specific cutaneous involvement in Whipple disease. ( 24562051 )
2014
17
Bilateral panuveitis associated with Whipple disease - case report. ( 27625936 )
2014
18
Primary Whipple disease of the CNS presenting with chorea and dystonia: A video case report. ( 29443151 )
2014
19
Pulmonary hypertension associated with Whipple disease. ( 25445953 )
2014
20
Neurocognitive impairment in Whipple disease with central nervous system involvement. ( 24674966 )
2014
21
Monoclonal B-cell lymphocyte proliferation in a patient presenting with historical Whipple disease. ( 23441662 )
2013
22
Rheumatic and musculoskeletal features of Whipple disease: a report of 29 cases. ( 24187107 )
2013
23
Oculomasticatory myorhythmia and agrypnia excitata guide the diagnosis of Whipple disease. ( 24210608 )
2013
24
Central nervous system involvement in Whipple disease: clinical study of 18 patients and long-term follow-up. ( 24145700 )
2013
25
A severe Whipple disease with an immune reconstitution inflammatory syndrome: An additional case of thalidomide efficiency. ( 24289960 )
2013
26
Whipple disease diagnosed with PCR using formalin-fixed paraffin-embedded specimens of the intestinal mucosa. ( 23318851 )
2013
27
Resolution of Whipple Disease-Induced Pulmonary Hypertension Following Antibiotic Therapy. ( 23344104 )
2013
28
Whipple disease revealed by lung involvement: a case report and literature review. ( 22670021 )
2012
29
Whipple disease mimicking adult-onset Still's disease and treated by anakinra: diagnosis using PCR. ( 22639877 )
2012
30
A unique posterior segment manifestation of whipple disease. ( 25390964 )
2012
31
Whipple disease a century after the initial description: increased recognition of unusual presentations, autoimmune comorbidities, and therapy effects. ( 22743287 )
2012
32
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
33
Whipple disease with crystalline keratopathy and chronic uveitis. ( 22965603 )
2012
34
Connecting the dots: the many systemic manifestations of whipple disease. ( 22347837 )
2012
35
Peripheral neuropathy in Whipples disease: a case report. ( 22716061 )
2012
36
Treated Whipple disease with erythema nodosum leprosum-like lesions: cutaneous PAS-positive macrophages slowly decrease with time and are associated with lymphangiectases: a case report. ( 22123268 )
2012
37
Whipple disease research accelerates. ( 21628651 )
2011
38
Infection: How should classic Whipple disease be managed? ( 20442732 )
2010
39
The immune reconstitution inflammatory syndrome in whipple disease: a cohort study. ( 21135294 )
2010
40
Whipple disease. ( 20224490 )
2010
41
Intestinal Mycobacterium avium complex infection initially misdiagnosed and mistreated as Whipple disease. ( 20138615 )
2010
42
Whipple disease of the central nervous system: an unusual occurrence in association with acquired immune deficiency syndrome. ( 19612977 )
2010
43
MR imaging of central nervous system Whipple disease: a 15-year review. ( 20395395 )
2010
44
The amnesic syndrome of primary Whipple disease of the brain. ( 20299864 )
2010
45
Isolated Whipple disease of the brain resembling a tumour. ( 19194653 )
2009
46
Renal amyloidosis in Whipple disease: a case report. ( 19918433 )
2009
47
Primary cerebral Whipple disease presenting as KlA1ver-Bucy syndrome. ( 19139312 )
2009
48
Whipple disease: unusual presentation of a protean and sometimes confusing disease. ( 18538828 )
2009
49
A paradoxical Tropheryma whipplei western blot differentiates patients with whipple disease from asymptomatic carriers. ( 19635029 )
2009
50
Reversible pulmonary hypertension in Whipple disease: a case report with clinicopathological implications, and literature review. ( 21686934 )
2009

Variations for Whipple Disease

Expression for Whipple Disease

Search GEO for disease gene expression data for Whipple Disease.

Pathways for Whipple Disease

Pathways related to Whipple Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.64 CD58 HLA-DQB1 HLA-DRB1
2 11.38 HLA-DQB1 HLA-DRB1 HSPD1
3 11.22 HLA-DQB1 HLA-DRB1
4 11.11 GRPEL1 HSPD1
5 11 HLA-DQB1 HLA-DRB1
6 10.96 CD58 HLA-DQB1 HLA-DRB1

GO Terms for Whipple Disease

Cellular components related to Whipple Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endocytic vesicle membrane GO:0030666 9.37 HLA-DQB1 HLA-DRB1
2 ER to Golgi transport vesicle membrane GO:0012507 9.32 HLA-DQB1 HLA-DRB1
3 transport vesicle membrane GO:0030658 9.26 HLA-DQB1 HLA-DRB1
4 clathrin-coated endocytic vesicle membrane GO:0030669 9.16 HLA-DQB1 HLA-DRB1
5 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DQB1 HLA-DRB1
6 MHC class II protein complex GO:0042613 8.62 HLA-DQB1 HLA-DRB1

Biological processes related to Whipple Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 viral process GO:0016032 9.58 HLA-DRB1 HSPD1 IL16
2 T cell costimulation GO:0031295 9.37 HLA-DQB1 HLA-DRB1
3 interferon-gamma-mediated signaling pathway GO:0060333 9.32 HLA-DQB1 HLA-DRB1
4 antigen processing and presentation GO:0019882 9.26 HLA-DQB1 HLA-DRB1
5 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.16 HLA-DQB1 HLA-DRB1
6 humoral immune response mediated by circulating immunoglobulin GO:0002455 8.96 HLA-DQB1 HLA-DRB1
7 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 8.62 HLA-DQB1 HLA-DRB1

Molecular functions related to Whipple Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 8.96 HLA-DQB1 HLA-DRB1
2 MHC class II receptor activity GO:0032395 8.62 HLA-DQB1 HLA-DRB1

Sources for Whipple Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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