MCID: WHP001
MIFTS: 50

Whipple Disease

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

Aliases & Classifications for Whipple Disease

MalaCards integrated aliases for Whipple Disease:

Name: Whipple Disease 12 72 49 55 41 14 69
Intestinal Lipodystrophy 12 72 49 50 55
Intestinal Lipophagic Granulomatosis 49 55 69
Secondary Non-Tropical Sprue 49 55
Whipple's Disease 12 50
Tropheryma Whippelii Infection 49
Whipples Disease 51

Characteristics:

Orphanet epidemiological data:

55
whipple disease
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe),1-9/1000000 (Italy); Age of onset: All ages;

Classifications:



Summaries for Whipple Disease

NINDS : 50 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, and has symptoms including hydrocephalus, proptosis and uveitis. 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.

NIH Rare Diseases : 49 Whipple disease is an infectious bacterial disease that affects many different organ systems and interferes with the body's ability to 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 malfunctions of the immune system. It is caused by infection from bacteria called Tropheryma whipplei (formerly called Tropheryma whippelii). When recognized and treated, Whipple disease can usually be cured. Untreated, the disease may be fatal. Last updated: 3/23/2017

Wikipedia : 72 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 67)
# Related Disease Score Top Affiliating Genes
1 hypersomnia 29.7 HLA-DQB1 HLA-DRB1
2 panuveitis 29.1 HLA-DQB1 HLA-DRB1 MRAP
3 limbic encephalitis with lgi1 antibodies 10.3 HLA-DQB1 HLA-DRB1
4 metal allergy 10.3 HLA-DQB1 HLA-DRB1
5 focal epithelial hyperplasia 10.3 HLA-DQB1 HLA-DRB1
6 beryllium disease 10.3 HLA-DQB1 HLA-DRB1
7 lichen planopilaris 10.3 HLA-DQB1 HLA-DRB1
8 type ii mixed cryoglobulinemia 10.3 HLA-DQB1 HLA-DRB1
9 idiopathic inflammatory myopathy 10.2 HLA-DQB1 HLA-DRB1
10 recurrent respiratory papillomatosis 10.2 HLA-DQB1 HLA-DRB1
11 actinic prurigo 10.2 CD58 HLA-DRB1
12 chronic beryllium disease 10.2 HLA-DQB1 HLA-DRB1
13 autoimmune pancreatitis 10.2 HLA-DQB1 HLA-DRB1
14 pityriasis rosea 10.2 HLA-DQB1 HLA-DRB1
15 lichen sclerosus 10.2 HLA-DQB1 HLA-DRB1
16 acute disseminated encephalomyelitis 10.2 HLA-DQB1 HLA-DRB1
17 pediatric multiple sclerosis 10.2 HLA-DQB1 HLA-DRB1
18 osteonecrosis of the jaw 10.2 HLA-DQB1 HLA-DRB1
19 microscopic colitis 10.2 HLA-DQB1 HLA-DRB1
20 idiopathic bronchiectasis 10.2 HLA-DQB1 HLA-DRB1
21 cystic echinococcosis 10.2 HLA-DQB1 HLA-DRB1
22 peanut allergy 10.2 HLA-DQB1 HLA-DRB1
23 oligoarticular juvenile idiopathic arthritis 10.2 HLA-DQB1 HLA-DRB1
24 geographic tongue 10.2 HLA-DQB1 HLA-DRB1
25 severe hemophilia a 10.2 HLA-DQB1 HLA-DRB1
26 ocular cicatricial pemphigoid 10.1 HLA-DQB1 HLA-DRB1
27 pelvic inflammatory disease 10.1 HLA-DQB1 HSPD1
28 paraneoplastic pemphigus 10.1 HLA-DQB1 HLA-DRB1
29 oral lichen planus 10.1 CD58 HSPD1
30 vogt-koyanagi-harada disease 10.1 HLA-DQB1 HLA-DRB1
31 urethritis 10.0 HSPD1 MRAP
32 mixed connective tissue disease 10.0 HLA-DRB1 HSPD1
33 cervicitis 10.0 HSPD1 MRAP
34 mesenteric lymphadenitis 10.0 HSPD1 MRAP
35 palindromic rheumatism 10.0 HLA-DRB1 MRAP
36 graft-versus-host disease 9.9 HLA-DQB1 HLA-DRB1
37 autoimmune hepatitis 9.9 HLA-DQB1 HLA-DRB1
38 pulmonary hypertension 9.9
39 arthritis 9.9
40 cerebritis 9.9
41 bone inflammation disease 9.8 HLA-DRB1 MRAP
42 diabetes mellitus, insulin-dependent 9.8 HLA-DQB1 HLA-DRB1 HSPD1
43 autoimmune disease 9.8 HLA-DQB1 HLA-DRB1 HSPD1
44 uveitis 9.8
45 bullous pemphigoid 9.7 HLA-DQB1 HLA-DRB1
46 multiple sclerosis 9.6 HLA-DQB1 HLA-DRB1 HSPD1
47 complement hyperactivation, angiopathic thrombosis, and protein-losing enteropathy 9.6
48 ataxia and polyneuropathy, adult-onset 9.6
49 aceruloplasminemia 9.6
50 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 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:

55 31 (show all 48)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 55 31 occasional (7.5%) Occasional (29-5%) HP:0000238
2 proptosis 55 31 occasional (7.5%) Occasional (29-5%) HP:0000520
3 uveitis 55 31 frequent (33%) Frequent (79-30%) HP:0000554
4 hypothyroidism 55 31 occasional (7.5%) Occasional (29-5%) HP:0000821
5 insulin resistance 55 31 occasional (7.5%) Occasional (29-5%) HP:0000855
6 seizures 55 31 occasional (7.5%) Occasional (29-5%) HP:0001250
7 ataxia 55 31 occasional (7.5%) Occasional (29-5%) HP:0001251
8 muscle weakness 55 31 occasional (7.5%) Occasional (29-5%) HP:0001324
9 myoclonus 55 31 hallmark (90%) Very frequent (99-80%) HP:0001336
10 arthritis 55 31 hallmark (90%) Very frequent (99-80%) HP:0001369
11 myocardial infarction 55 31 occasional (7.5%) Occasional (29-5%) HP:0001658
12 pericarditis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001701
13 splenomegaly 55 31 frequent (33%) Frequent (79-30%) HP:0001744
14 anemia 55 31 occasional (7.5%) Occasional (29-5%) HP:0001903
15 fever 55 31 hallmark (90%) Very frequent (99-80%) HP:0001945
16 polydipsia 55 31 occasional (7.5%) Occasional (29-5%) HP:0001959
17 diarrhea 55 31 hallmark (90%) Very frequent (99-80%) HP:0002014
18 malabsorption 55 31 hallmark (90%) Very frequent (99-80%) HP:0002024
19 abdominal pain 55 31 hallmark (90%) Very frequent (99-80%) HP:0002027
20 anorexia 55 31 hallmark (90%) Very frequent (99-80%) HP:0002039
21 respiratory insufficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0002093
22 pleuritis 55 31 frequent (33%) Frequent (79-30%) HP:0002102
23 gastrointestinal hemorrhage 55 31 occasional (7.5%) Occasional (29-5%) HP:0002239
24 hepatomegaly 55 31 frequent (33%) Frequent (79-30%) HP:0002240
25 sleep disturbance 55 31 frequent (33%) Frequent (79-30%) HP:0002360
26 developmental regression 55 31 hallmark (90%) Very frequent (99-80%) HP:0002376
27 encephalitis 55 31 occasional (7.5%) Occasional (29-5%) HP:0002383
28 increased intracranial pressure 55 31 occasional (7.5%) Occasional (29-5%) HP:0002516
29 hypotension 55 31 frequent (33%) Frequent (79-30%) HP:0002615
30 arthralgia 55 31 hallmark (90%) Very frequent (99-80%) HP:0002829
31 hyponatremia 55 31 occasional (7.5%) Occasional (29-5%) HP:0002902
32 myalgia 55 31 frequent (33%) Frequent (79-30%) HP:0003326
33 cachexia 55 31 hallmark (90%) Very frequent (99-80%) HP:0004326
34 cranial nerve paralysis 55 31 occasional (7.5%) Occasional (29-5%) HP:0006824
35 abnormal pyramidal signs 55 31 occasional (7.5%) Occasional (29-5%) HP:0007256
36 generalized hyperpigmentation 55 31 occasional (7.5%) Occasional (29-5%) HP:0007440
37 peripheral neuropathy 55 31 occasional (7.5%) Occasional (29-5%) HP:0009830
38 edema of the lower limbs 55 31 occasional (7.5%) Occasional (29-5%) HP:0010741
39 fatigue 55 31 hallmark (90%) Very frequent (99-80%) HP:0012378
40 cough 55 31 occasional (7.5%) Occasional (29-5%) HP:0012735
41 myocarditis 55 31 occasional (7.5%) Occasional (29-5%) HP:0012819
42 myositis 55 31 occasional (7.5%) Occasional (29-5%) HP:0100614
43 erectile abnormalities 55 31 occasional (7.5%) Occasional (29-5%) HP:0100639
44 mediastinal lymphadenopathy 55 31 hallmark (90%) Very frequent (99-80%) HP:0100721
45 chest pain 55 31 occasional (7.5%) Occasional (29-5%) HP:0100749
46 galactorrhea 55 31 occasional (7.5%) Occasional (29-5%) HP:0100829
47 depression 55 Very frequent (99-80%)
48 depressivity 31 hallmark (90%) HP:0000716

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

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:

38
Brain, Lung, Eye, Heart, Skin, Spinal Cord, B Cells

Publications for Whipple Disease

Articles related to Whipple Disease:

(show top 50) (show all 108)
# Title Authors Year
1
A case of rapidly progressive dementia: Whipple disease of CNS. ( 29079886 )
2017
2
Whipple Disease ( 28722966 )
2017
3
Cytology of cerebrospinal fluid in CNS Whipple disease. ( 28770535 )
2017
4
Genotypic analysis of Tropheryma whipplei from patients with Whipple disease in the Americas. ( 28385924 )
2017
5
Ultrastructural changes of small intestine in Whipple disease patient. ( 28230475 )
2017
6
Deep Vein Thrombosis as Initial Manifestation of Whipple Disease. ( 27920655 )
2016
7
Whipple disease confined to the central nervous system presenting as a cystic tumor: Case report and review of literature. ( 27593833 )
2016
8
Pseudo-Whipple Disease Cutaneous Lesions. ( 27097339 )
2016
9
Prosopagnosia as the Presenting Symptom of Whipple Disease. ( 27336807 )
2016
10
Gastrointestinal diagnosis of classical whipple disease: clinical, endoscopic, and histopathologic features in 191 patients. ( 25881849 )
2015
11
Primary Whipple disease of the brain: case report with long-term clinical and MRI follow-up. ( 26445540 )
2015
12
Atypical presentation of Whipple disease with no diarrhea. ( 26275278 )
2015
13
Neurocognitive impairment in Whipple disease with central nervous system involvement. ( 24674966 )
2014
14
Bilateral panuveitis associated with Whipple disease - case report. ( 27625936 )
2014
15
Specific cutaneous involvement in Whipple disease. ( 24562051 )
2014
16
Pulmonary hypertension associated with Whipple disease. ( 25445953 )
2014
17
Primary Whipple disease of the CNS presenting with chorea and dystonia: A video case report. ( 29443151 )
2014
18
A severe Whipple disease with an immune reconstitution inflammatory syndrome: An additional case of thalidomide efficiency. ( 24289960 )
2013
19
Oculomasticatory myorhythmia and agrypnia excitata guide the diagnosis of Whipple disease. ( 24210608 )
2013
20
Monoclonal B-cell lymphocyte proliferation in a patient presenting with historical Whipple disease. ( 23441662 )
2013
21
Whipple disease diagnosed with PCR using formalin-fixed paraffin-embedded specimens of the intestinal mucosa. ( 23318851 )
2013
22
Resolution of Whipple Disease-Induced Pulmonary Hypertension Following Antibiotic Therapy. ( 23344104 )
2013
23
Rheumatic and musculoskeletal features of Whipple disease: a report of 29 cases. ( 24187107 )
2013
24
Central nervous system involvement in Whipple disease: clinical study of 18 patients and long-term follow-up. ( 24145700 )
2013
25
A unique posterior segment manifestation of whipple disease. ( 25390964 )
2012
26
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
27
Whipple disease with crystalline keratopathy and chronic uveitis. ( 22965603 )
2012
28
Whipple disease a century after the initial description: increased recognition of unusual presentations, autoimmune comorbidities, and therapy effects. ( 22743287 )
2012
29
Whipple disease revealed by lung involvement: a case report and literature review. ( 22670021 )
2012
30
Peripheral neuropathy in Whipples disease: a case report. ( 22716061 )
2012
31
Whipple disease mimicking adult-onset Still's disease and treated by anakinra: diagnosis using PCR. ( 22639877 )
2012
32
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
33
Connecting the dots: the many systemic manifestations of whipple disease. ( 22347837 )
2012
34
Whipple disease research accelerates. ( 21628651 )
2011
35
Infection: How should classic Whipple disease be managed? ( 20442732 )
2010
36
Whipple disease. ( 20224490 )
2010
37
The amnesic syndrome of primary Whipple disease of the brain. ( 20299864 )
2010
38
Whipple disease of the central nervous system: an unusual occurrence in association with acquired immune deficiency syndrome. ( 19612977 )
2010
39
Intestinal Mycobacterium avium complex infection initially misdiagnosed and mistreated as Whipple disease. ( 20138615 )
2010
40
MR imaging of central nervous system Whipple disease: a 15-year review. ( 20395395 )
2010
41
The immune reconstitution inflammatory syndrome in whipple disease: a cohort study. ( 21135294 )
2010
42
Renal amyloidosis in Whipple disease: a case report. ( 19918433 )
2009
43
A paradoxical Tropheryma whipplei western blot differentiates patients with whipple disease from asymptomatic carriers. ( 19635029 )
2009
44
Reversible pulmonary hypertension in Whipple disease: a case report with clinicopathological implications, and literature review. ( 21686934 )
2009
45
Isolated Whipple disease of the brain resembling a tumour. ( 19194653 )
2009
46
Whipple disease: unusual presentation of a protean and sometimes confusing disease. ( 18538828 )
2009
47
Postoperative panophthalmitis caused by Whipple disease. ( 19402983 )
2009
48
Primary cerebral Whipple disease presenting as KlA1ver-Bucy syndrome. ( 19139312 )
2009
49
Recurrent orbital inflammation and Whipple disease. ( 18379941 )
2008
50
Neurologically presenting Whipple disease: case report and review of the literature. ( 18381382 )
2008

Variations for Whipple Disease

Expression for Whipple Disease

Search GEO for disease gene expression data for Whipple Disease.

Pathways for Whipple Disease

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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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