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MCID: HYP074
MIFTS: 53

Hypersensitivity Vasculitis

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Immune diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Hypersensitivity Vasculitis

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MalaCards integrated aliases for Hypersensitivity Vasculitis:

Name: Hypersensitivity Vasculitis 12 20 15 17
Cutaneous Small Vessel Vasculitis 20 58 70
Vasculitis, Leukocytoclastic, Cutaneous 44 70
Hypersensitivity Angiitis 20 32
Cutaneous Leukocytoclastic Vasculitis 20
Cutaneous Hypersensitivity Vasculitis 58
Cutaneous Leukocytoclastic Angiitis 20
Drug Induced Cutaneous Vasculitis 70
Vasculitis Hypersensitivity 73
Leukocytoclastic Vasculitis 70
Leukocytoclastic Angiitis 20

Characteristics:

Orphanet epidemiological data:

58
cutaneous small vessel vasculitis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Cardiovascular diseases Bone diseases Immune diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 32 33
Orphanet: 58  
Rare circulatory system diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:9809
ICD9CM 34 446.2
MeSH 44 D018366
NCIt 50 C35119
SNOMED-CT 67 195350001
ICD10 32 M31.0
ICD10 via Orphanet 33 M31.0
UMLS via Orphanet 71 C0151436
Orphanet 58 ORPHA889
UMLS 70 C0151436 C2973529 C3812646 more
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Summaries for Hypersensitivity Vasculitis

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GARD : 20 Hypersensitivity vasculitis is an extreme reaction to a drug, infection, or foreign substance that leads to inflammation and damage to blood vessels of the skin. Signs and symptoms may include purple-colored spots and patches on the skin; skin lesions on the legs, buttocks, or trunk; blisters on the skin; hives ( urticaria ); and/or open sores with dead tissue (necrotic ulcers ). This condition is caused by an allergic reaction to a drug or other foreign substance. This condition usually goes away over time; but on occasion, people can have repeated episodes.

MalaCards based summary : Hypersensitivity Vasculitis, also known as cutaneous small vessel vasculitis, is related to allergic cutaneous vasculitis and allergic disease, and has symptoms including purpura An important gene associated with Hypersensitivity Vasculitis is PRTN3 (Proteinase 3), and among its related pathways/superpathways are Innate Immune System and Allograft rejection. The drugs Levoleucovorin and Dapsone have been mentioned in the context of this disorder. Affiliated tissues include skin, neutrophil and colon, and related phenotypes are fever and myalgia

Disease Ontology : 12 A hypersensitivity reaction type III disease that is characterized by inflammation and injury to blood vessels and has symptom purpura, telangiectasia, blistering, urticaria, and ulcers.

Wikipedia : 73 Cutaneous small-vessel vasculitis (CSVV), also known as hypersensitivity vasculitis, cutaneous... more...

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Related Diseases for Hypersensitivity Vasculitis

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Diseases related to Hypersensitivity Vasculitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 686)
# Related Disease Score Top Affiliating Genes
1 allergic cutaneous vasculitis 32.3 PRTN3 MPO
2 allergic disease 31.0 TNF MPO IL6 ICOSLG
3 goodpasture syndrome 30.7 PRTN3 MPO ALB
4 subacute bacterial endocarditis 30.5 PRTN3 MPO CRP
5 cardiac tamponade 30.4 CRP ALB ACE
6 microscopic polyangiitis 30.3 MPO FCGR3B
7 pulmonary tuberculosis 30.3 TNF IL6 C3 ALB
8 vasculitis 30.2 TNF SERPINA3 PRTN3 MPO MEFV IL6
9 pyoderma 30.2 TNF MEFV CRP
10 lymphadenitis 30.1 TNF SERPINA3 ICOSLG CRP
11 sarcoidosis 1 30.1 TNF SERPINA3 ICOSLG CRP ACE
12 orchitis 30.1 TNF IL6 CRP
13 neuroretinitis 30.1 CRP ACE
14 antiphospholipid syndrome 30.1 TNF CRP C3
15 urticaria 30.1 TNF IL6 CRP ACE
16 ulcerative colitis 30.1 TNF PRTN3 MPO IL6 CRP
17 churg-strauss syndrome 30.0 TNF PRTN3 MPO
18 iron deficiency anemia 30.0 TNF IL6 CRP
19 disseminated intravascular coagulation 30.0 TNF IL6 CRP
20 purpura 30.0 TNF MEFV IL6 CRP C3 ACE
21 infective endocarditis 30.0 TNF IL6 CRP
22 periodontitis 29.9 TNF SERPINA3 IL6 CRP
23 retinal vein occlusion 29.9 TNF IL6 ACE
24 mononeuritis multiplex 29.9 TNF PRTN3 MPO CRP ACE
25 salmonellosis 29.9 TNF IL6 ALB
26 spondylitis 29.9 TNF IL6 ICOSLG CRP
27 autoimmune vasculitis 29.9 TNF PRTN3 MPO IL6 CRP
28 iritis 29.9 TNF IL6 CRP ACE
29 pericardial effusion 29.9 PRTN3 IL6 CRP ALB ACE
30 extrinsic allergic alveolitis 29.8 MPO IL6 ALB
31 endocarditis 29.8 TNF IL6 CRP ALB
32 erythema nodosum 29.8 TNF IL6 CRP C3
33 aphthous stomatitis 29.8 TNF MEFV IL6 ACE
34 subacute thyroiditis 29.8 TNF IL6 CRP
35 thyroiditis 29.8 TNF IL6 CRP
36 granulomatous hepatitis 29.8 TNF CRP ALB ACE
37 mononeuropathy 29.7 TNF PRTN3 MPO CRP ALB
38 leukemia, acute monocytic 29.7 TNF MPO IL6
39 polyarteritis nodosa 29.7 PRTN3 MPO MEFV IL6 CRP
40 alpha-1-antitrypsin deficiency 29.7 TNF SERPINA3 PRTN3 MPO ALB
41 acute myocardial infarction 29.6 SERPINA3 IL6 CRP ACE
42 hepatic encephalopathy 29.6 TNF IL6 ALB
43 gastroenteritis 29.6 TNF IL6 CRP ALB
44 temporal arteritis 29.6 TNF SERPINA3 PRTN3 MPO IL6 CRP
45 polyneuropathy 29.6 TNF IL6 CRP ALB ACE
46 syphilis 29.5 TNF SERPINA3 PRTN3 IL6 CRP ALB
47 bilirubin metabolic disorder 29.5 TNF IL6 CRP ALB
48 henoch-schoenlein purpura 29.5 SERPINA3 PRTN3 MPO MIR98 MEST MEFV
49 pulmonary fibrosis, idiopathic 29.5 TNF MPO IL6 CRP ALB ACE
50 bronchiectasis 29.5 TNF SERPINA3 MPO IL6 CRP

Comorbidity relations with Hypersensitivity Vasculitis via Phenotypic Disease Network (PDN):


Acute Cystitis Deficiency Anemia
Heart Disease Postinflammatory Pulmonary Fibrosis
Rheumatoid Arthritis Systemic Lupus Erythematosus

Graphical network of the top 20 diseases related to Hypersensitivity Vasculitis:



Diseases related to Hypersensitivity Vasculitis
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Symptoms & Phenotypes for Hypersensitivity Vasculitis

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Human phenotypes related to Hypersensitivity Vasculitis:

58 31 (show all 14)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 58 31 hallmark (90%) Very frequent (99-80%) HP:0001945
2 myalgia 58 31 hallmark (90%) Very frequent (99-80%) HP:0003326
3 purpura 58 31 hallmark (90%) Very frequent (99-80%) HP:0000979
4 erythema 58 31 hallmark (90%) Very frequent (99-80%) HP:0010783
5 urticaria 58 31 hallmark (90%) Very frequent (99-80%) HP:0001025
6 vasculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002633
7 papule 58 31 hallmark (90%) Very frequent (99-80%) HP:0200034
8 cutis marmorata 58 31 hallmark (90%) Very frequent (99-80%) HP:0000965
9 gangrene 58 31 hallmark (90%) Very frequent (99-80%) HP:0100758
10 recurrent skin infections 58 31 hallmark (90%) Very frequent (99-80%) HP:0001581
11 arthralgia 58 31 frequent (33%) Frequent (79-30%) HP:0002829
12 skin rash 58 31 frequent (33%) Frequent (79-30%) HP:0000988
13 subcutaneous nodule 58 31 occasional (7.5%) Occasional (29-5%) HP:0001482
14 abnormal oral cavity morphology 58 31 occasional (7.5%) Occasional (29-5%) HP:0000163

Symptoms:

12
  • • purpura

MGI Mouse Phenotypes related to Hypersensitivity Vasculitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.4 ACE ALB C1GALT1 C3 CRP FCGR3B
Sources

Drugs & Therapeutics for Hypersensitivity Vasculitis

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Drugs for Hypersensitivity Vasculitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 42)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Levoleucovorin Approved, Investigational Phase 4 68538-85-2 149436
2
Dapsone Approved, Investigational Phase 4 80-08-0 2955
3
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
4 Anti-Infective Agents Phase 4
5 Vitamin B9 Phase 4
6 Folic Acid Antagonists Phase 4
7 Antimalarials Phase 4
8 Vitamin B Complex Phase 4
9 Antiparasitic Agents Phase 4
10 Folate Phase 4
11 Anti-Bacterial Agents Phase 4
12 Antiprotozoal Agents Phase 4
13
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
14
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
15
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
16
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
17
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
18
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
19 Neuroprotective Agents Phase 2
20 Gastrointestinal Agents Phase 2
21 Methylprednisolone Acetate Phase 2
22 glucocorticoids Phase 2
23 Hormone Antagonists Phase 2
24 Antineoplastic Agents, Hormonal Phase 2
25 Hormones Phase 2
26 Antiemetics Phase 2
27 Anti-Inflammatory Agents Phase 2
28 Protective Agents Phase 2
29
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
30
Lenograstim Approved, Investigational 135968-09-1
31
Prednisone Approved, Vet_approved 53-03-2 5865
32
Clotrimazole Approved, Vet_approved 23593-75-1 2812
33
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
34 Immunosuppressive Agents
35 Alkylating Agents
36 Cyclosporins
37 Immunologic Factors
38 Antirheumatic Agents
39 Dermatologic Agents
40 Antilymphocyte Serum
41 Calcineurin Inhibitors
42 Antifungal Agents

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase IV, Randomised, Multicentre, Double-blind, Study to Evaluate the Clinical Utility of Prospective Genetic Screening (HLA-B*1301) for Susceptibility to Dapsone Hypersensitivity Syndrome Unknown status NCT02550080 Phase 4 Dapsone
2 Phase II Study of Prednisolone/Methylprednisolone Absorption in Children With Juvenile Dermatomyositis Completed NCT00004357 Phase 2 Methylprednisolone;Prednisolone
3 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055 anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone

Search NIH Clinical Center for Hypersensitivity Vasculitis

Cochrane evidence based reviews: vasculitis, leukocytoclastic, cutaneous

Sources

Genetic Tests for Hypersensitivity Vasculitis

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Anatomical Context for Hypersensitivity Vasculitis

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MalaCards organs/tissues related to Hypersensitivity Vasculitis:

40
Skin, Neutrophil, Colon, Prostate, Endothelial
Sources

Publications for Hypersensitivity Vasculitis

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Articles related to Hypersensitivity Vasculitis:

(show top 50) (show all 310)
# Title Authors PMID Year
1
Low utility of radiologic imaging in evaluating cutaneous small-vessel vasculitis: A multi-institutional retrospective study. 61
33011319 2021
2
Checkpoint Inhibitor-associated Cutaneous Small Vessel Vasculitis: A Review of the Literature. 61
33323871 2021
3
Case for diagnosis. Cutaneous small vessel vasculitis (anti-proteinase 3 positive), fever, hemoptysis, and lung cavitation in an adult. 61
33579583 2021
4
Exuberant bullous vasculitis associated with SARS-CoV-2 infection. 61
33457205 2021
5
Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review. 61
33401725 2021
6
Complementary Use of U.S. FDA's Adverse Event Reporting System and Sentinel System to Characterize Direct Oral Anticoagulants-Associated Cutaneous Small Vessel Vasculitis. 61
33090530 2020
7
Lessons From the First Wave of the Pandemic: Skin Features of COVID-19 can be Divided Into Inflammatory and Vascular Patterns. 61
33179534 2020
8
Cutaneous small-vessel vasculitis associated with novel 2019 coronavirus SARS-CoV-2 infection (COVID-19). 61
32453865 2020
9
Widespread cutaneous small vessel vasculitis secondary to COVID-19 infection. 61
32880974 2020
10
Cutaneous small vessel vasculitis secondary to COVID-19 infection: a case report. 61
32441876 2020
11
Hypocomplementemic cutaneous small vessel vasculitis in a patient with IgG4-related disease. 61
33147668 2020
12
Posterior reversible encephalopathy syndrome associated with Henoch Schonlein Purpura in a pediatric patient. 61
33097321 2020
13
Neutrophil Extracellular Traps are Present in Immune-complex-mediated Cutaneous Small Vessel Vasculitis and Correlate with the Production of Reactive Oxygen Species and the Severity of Vessel Damage. 61
31663600 2020
14
Cutaneous vasculitis in SLE. 61
32963114 2020
15
Cutaneous arteriolitis: A novel cutaneous small vessel vasculitis disorder clinicopathologically different from cutaneous polyarteritis nodosa and cutaneous venulitis. 61
32338386 2020
16
Assessing practice gaps in the outpatient management of cutaneous small vessel vasculitis. 61
31962090 2020
17
Clinical and therapeutic overlap of pyoderma gangrenosum, cutaneous small vessel vasculitis, and immunoglobulin A. 61
32118287 2020
18
Atypical vasculopathic varicella-zoster infection mimicking cutaneous small-vessel vasculitis. 61
32052407 2020
19
Protocol for a randomized multicenter study for isolated skin vasculitis (ARAMIS) comparing the efficacy of three drugs: azathioprine, colchicine, and dapsone. 61
32345372 2020
20
Diffuse cutaneous reaction following PPV-23 pneumococcal vaccine: an immunisation-associated hypersensitivity vasculitis. 61
32193184 2020
21
Warfarin induced leukocytoclastic vasculitis: an extraordinary side effect. 61
31375992 2020
22
102 WITHDRAWN: Cutaneous Small Vessel Vasculitis in Two Patients With Left Ventricular Assist Devices. 61
33279146 2020
23
Cutaneous small-vessel vasculitis following hysterosalpingo-foam sonography (HyFoSy). 61
31219636 2019
24
An Uncommon Coexistence of Sarcoidosis and Cutaneous Leukocytoclastic Vasculitis in an Adult. 61
31896849 2019
25
Cutaneous small-vessel vasculitis associated with paediatric ulcerative colitis: A case study and literature review. 61
30895688 2019
26
Alcohol-Associated Immunoglobulin A Vasculitis: A Case Report and Review of the Literature. 61
32596212 2019
27
A case of dolutegravir-induced cutaneous small vessel vasculitis. 61
31356258 2019
28
The color of skin: purple diseases of the skin, nails, and mucosa. 61
31896408 2019
29
Androgen deprivation therapy for prostate cancer and the risk of autoimmune diseases. 61
30692587 2019
30
Cutaneous necrotizing small-vessel vasculitis induced by acute hepatitis E. 61
31428384 2019
31
Henoch-Schonlein purpura associated with HLA-B27 positive axial spondyloarthritis in a young man. 61
31129640 2019
32
Autism and Piperacillin-tazobactam-induced Hypersensitivity Vasculitis: A Silent Malady. 61
31281757 2019
33
A rare association of cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) and hypersensitivity pneumonia (extrinsic allergic alveolitis) in a pigeon breeder - case report and literature review. 61
31263863 2019
34
A Unique Case Report on Hypersensitivity Vasculitis as an Allergic Reaction to the Herpes Zoster Vaccine. 61
30122132 2019
35
Role of Direct Immunofluorescence in Cutaneous Small-Vessel Vasculitis: Experience From a Tertiary Center. 61
29672364 2018
36
Checkpoint inhibitor-associated cutaneous small vessel vasculitis. 61
30112452 2018
37
Paraneoplastic cutaneous small-vessel vasculitis as a presentation of recurrent metastatic breast cancer. 61
29984288 2018
38
Unusual presentation of erythema elevatum diutinum with underlying hepatitis B infection. 61
30063775 2018
39
[Extra-facial Lever granuloma on a herpes zoster scar: Wolf's isotopic response]. 61
29673750 2018
40
Propylthiouracil-induced ANCA-negative cutaneous small vessel vasculitis. 61
29441165 2018
41
Pediatric vasculitis: a single center experience. 61
28929493 2017
42
Cutaneous Small-Vessel Vasculitis in Two Children with Inflammatory Bowel Disease: Case Series and Review of the Literature. 61
28771805 2017
43
Successful Treatment of Cutaneous Small Vessel Vasculitis With Leflunomide. 61
28614577 2017
44
Incidence of autoimmune diseases in patients with scabies: a nationwide population-based study in Taiwan. 61
28421357 2017
45
Cefazolin as a cause of leukocytoclastic vasculitis. 61
28588872 2017
46
Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV). 61
28328827 2017
47
Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases. 61
27580419 2017
48
Premature diagnosis of calciphylaxis without pathological indications finally diagnosed as cutaneous small-vessel vasculitis: a post-mortem case report. 61
30549918 2017
49
Acrally distributed dermatoses: Vascular dermatoses (purpura and vasculitis). 61
27938815 2017
50
Approach to cutaneous vasculitides with special emphasis on small vessel vasculitis: histopathology and direct immunofluorescence. 61
27787337 2017
Sources

Variations for Hypersensitivity Vasculitis

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Expression for Hypersensitivity Vasculitis

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Search GEO for disease gene expression data for Hypersensitivity Vasculitis.
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Pathways for Hypersensitivity Vasculitis

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Pathways related to Hypersensitivity Vasculitis according to GeneCards Suite gene sharing:

(show all 11)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 65
Show member pathways
 13.74 TNF SERPINA3 PRTN3 MPO MEFV IL6
2
Allograft rejection 1 36
Show member pathways
 12.63 TNF IL6 ICOSLG C4A C3
3
Tuberculosis 36
12.01 TNF IL6 FCGR3B C3
4
Phagosome 36
11.89 MPO FCGR3B FCAR C3
5
Staphylococcus aureus infection 36
11.6 FCGR3B FCAR C4A C3
6
Folate Metabolism 1
Show member pathways
 11.43 TNF SERPINA3 MPO IL6 CRP ALB
7
Legionellosis 36
11.41 TNF IL6 C3
8
Pertussis 36
11.34 TNF IL6 C4A C3
9
amb2 Integrin signaling 1
11.29 TNF MPO IL6
10
IgA-Producing B Cells in the Intestine 64
11.08 TNF IL6 ICOSLG FCGR3B
11
Overview of nanoparticle effects 1
10.71 TNF IL6 CRP
Sources

GO Terms for Hypersensitivity Vasculitis

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Cellular components related to Hypersensitivity Vasculitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.93 SERPINA3 PRTN3 MPO MEST JCHAIN ICOSLG
2 extracellular space GO:0005615 9.73 TNF SERPINA3 PRTN3 MPO MIR98 JCHAIN
3 blood microparticle GO:0072562 9.65 SERPINA3 JCHAIN C4A C3 ALB
4 azurophil granule lumen GO:0035578 9.62 SERPINA3 PRTN3 MPO C3
5 extracellular region GO:0005576 9.47 TNF SERPINA3 PRTN3 MPO JCHAIN IL6

Biological processes related to Hypersensitivity Vasculitis according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.8 IL6 C4A C3 ALB
2 immune response GO:0006955 9.8 TNF JCHAIN IL6 FCGR3B FCAR C3
3 neutrophil degranulation GO:0043312 9.73 SERPINA3 PRTN3 MPO FCGR3B FCAR C3
4 humoral immune response GO:0006959 9.63 TNF JCHAIN IL6
5 acute-phase response GO:0006953 9.58 SERPINA3 IL6 CRP
6 vascular endothelial growth factor production GO:0010573 9.48 TNF IL6
7 positive regulation of apoptotic cell clearance GO:2000427 9.43 C4A C3
8 O-glycan processing, core 1 GO:0016267 9.37 C1GALT1C1 C1GALT1
9 neutrophil mediated immunity GO:0002446 9.33 IL6 FCAR ACE
10 inflammatory response GO:0006954 9.17 TNF SERPINA3 MEFV IL6 CRP C4A
11 negative regulation of lipid storage GO:0010888 9.13 TNF IL6 CRP

Molecular functions related to Hypersensitivity Vasculitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 complement component C1q binding GO:0001849 9.16 CRP C4A
2 IgA binding GO:0019862 8.96 JCHAIN FCAR
3 glycoprotein-N-acetylgalactosamine 3-beta-galactosyltransferase activity GO:0016263 8.62 C1GALT1C1 C1GALT1
Sources

Sources for Hypersensitivity Vasculitis

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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