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MCID: HYP074
MIFTS: 56

Hypersensitivity Vasculitis

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Immune diseases, Rare diseases
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Aliases & Classifications for Hypersensitivity Vasculitis

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MalaCards integrated aliases for Hypersensitivity Vasculitis:

Name: Hypersensitivity Vasculitis 11 19 14 16 33
Cutaneous Small Vessel Vasculitis 19 58 71
Hypersensitivity Angiitis 19 31 33
Vasculitis, Leukocytoclastic, Cutaneous 43 71
Cutaneous Leukocytoclastic Vasculitis 19 33
Leukocytoclastic Vasculitis 71 33
Leukocytoclastic Angiitis 19 75
Cutaneous Hypersensitivity Vasculitis 58
Cutaneous Leukocytoclastic Angiitis 19
Drug Induced Cutaneous Vasculitis 71
Cutaneous Small-Vessel Vasculitis 75
Vasculitis Hypersensitivity 75
Allergic Vasculitis 33

Characteristics:


Prevelance:

Cutaneous Small Vessel Vasculitis: 1-9/100000 (Norway) 58

Age Of Onset:

Cutaneous Small Vessel Vasculitis: All ages 58

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Immune diseases Cardiovascular diseases Bone diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 31 32
ICD11: 33
Orphanet: 58  
Rare circulatory system diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 11 DOID:9809
ICD9CM 34 446.2
MeSH 43 D018366
NCIt 49 C35119
SNOMED-CT 68 195350001
ICD10 31 M31.0
ICD10 via Orphanet 32 M31.0
UMLS via Orphanet 72 C0151436
Orphanet 58 ORPHA889
UMLS 71 C0151436 C2973529 C3812646 more
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Summaries for Hypersensitivity Vasculitis

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GARD: 19 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 Hematopoietic Stem Cells and Lineage-specific Markers. The drugs Levoleucovorin and Dapsone have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and neutrophil, and related phenotypes are fever and myalgia

Orphanet: 58 A small vessel vasculitis characterized by neutrophilic inflammation predominantly limited to the superficial cutaneous postcapillary venules and without systemic vasculitis or glomerulonephritis. Typical presentation is of unifocal or multifocal palpable purpura on the lower extremities.

Disease Ontology: 11 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: 75 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 1095)
# Related Disease Score Top Affiliating Genes
1 allergic cutaneous vasculitis 32.3 PRTN3 MPO
2 allergic disease 30.7 TNF SERPINA3 IL6 ICOSLG CCR6
3 cryoglobulinemia 30.7 TNF IL6 CD79A
4 purpura 30.4 TNF IL6 CRP CD79A C3
5 sjogren syndrome 30.4 PRTN3 ICOSLG FCGR3B CD79A CCR6
6 erythema elevatum diutinum 30.4 PRTN3 MPO FCAR CRP
7 maxillary sinusitis 30.3 PRTN3 MPO CRP
8 secondary syphilis 30.3 TNF CRP ACE
9 subacute bacterial endocarditis 30.3 PRTN3 MPO CRP ALB
10 pulmonary tuberculosis 30.3 TNF IL6 CRP C3 ALB
11 salmonellosis 30.3 TNF IL6 CCR6
12 tuberculous peritonitis 30.2 CRP ALB
13 hepatic infarction 30.2 CFHR2 ALB
14 mononeuritis multiplex 30.2 TNF PRTN3 MPO CRP
15 cogan syndrome 30.2 TNF PRTN3 CRP
16 igg4-related disease 30.2 PRTN3 MPO ICOSLG CRP CCR6
17 retinal vasculitis 30.2 TNF CCR6 ACE
18 severe acute respiratory syndrome 30.2 TNF IL6 CRP ACE
19 periapical periodontitis 30.2 TNF IL6 ICOSLG
20 splenic infarction 30.2 PRTN3 MPO CRP ALB
21 antiphospholipid syndrome 30.2 TNF CRP CD79A C3
22 autoimmune pancreatitis 30.2 ICOSLG CRP CCR6
23 pyoderma gangrenosum 30.2 TNF MEFV CCR6
24 thrombophlebitis 30.2 SERPINA3 CRP ALB
25 hemorrhagic disease 30.2 PRTN3 MPO CRP ALB
26 pancytopenia 30.2 MPO IL6 CRP ALB ACE
27 neutrophilic dermatosis, acute febrile 30.2 TNF SERPINA3 MPO MEFV CRP
28 drug allergy 30.2 CRP ALB ACE
29 cat-scratch disease 30.1 PRTN3 MPO CRP ACE
30 duodenitis 30.1 TNF CRP ALB
31 cellulitis 30.1 TNF IL6 CRP CCR6 ALB
32 urticaria 30.1 TNF IL6 CRP ACE
33 viral hepatitis 30.1 MPO IL6 CRP ALB
34 mitral valve insufficiency 30.1 CRP ALB ACE
35 miliary tuberculosis 30.1 TNF CRP ALB ACE
36 vasculitis 30.1 TNF SERPINA3 PRTN3 MPO MEFV IL6
37 microscopic polyangiitis 30.1 PRTN3 MPO
38 granulomatous dermatitis 30.1 TNF CRP ACE
39 cryptococcosis 30.1 TNF FCGR3B CCR6
40 brucellosis 30.1 TNF MPO MEFV IL6 CRP
41 oligohydramnios 30.1 CRP ALB ACE
42 iron deficiency anemia 30.1 TNF IL6 CRP
43 osteomyelitis 30.1 TNF MEFV IL6 CRP
44 food allergy 30.1 TNF ICOSLG CCR6
45 goodpasture syndrome 30.1 PRTN3 MPO CRP CFHR2 CCR6 C3
46 castleman disease 30.1 IL6 CRP CCR6
47 churg-strauss syndrome 30.1 TNF PRTN3 MPO
48 pyoderma 30.1 TNF MEFV CRP CCR6
49 neutropenia 30.1 TNF MPO IL6 FCGR3B CRP ALB
50 disseminated intravascular coagulation 30.1 TNF 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 30 (show all 14)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0001945
2 myalgia 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0003326
3 purpura 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0000979
4 erythema 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0010783
5 urticaria 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0001025
6 vasculitis 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0002633
7 papule 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0200034
8 cutis marmorata 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0000965
9 gangrene 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0100758
10 recurrent skin infections 58 30 Hallmark (90%) Very frequent (99-80%)
 HP:0001581
11 arthralgia 58 30 Frequent (33%) Frequent (79-30%)
 HP:0002829
12 skin rash 58 30 Frequent (33%) Frequent (79-30%)
 HP:0000988
13 subcutaneous nodule 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0001482
14 abnormal oral cavity morphology 58 30 Occasional (7.5%) Occasional (29-5%)
 HP:0000163

Symptoms:

11
  • • purpura

MGI Mouse Phenotypes related to Hypersensitivity Vasculitis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.1 ACE ALB C1GALT1 C3 CCR6 CD79A
2 cardiovascular system MP:0005385 9.9 ACE ALB C1GALT1 C1GALT1C1 C3 CCR6
3 immune system MP:0005387 9.83 ACE ALB C1GALT1 C3 CCR6 CD79A
4 hematopoietic system MP:0005397 9.44 ACE C1GALT1 C3 CCR6 CD79A FCGR3B
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Drugs & Therapeutics for Hypersensitivity Vasculitis

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

(show all 43)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Levoleucovorin Approved, Experimental, Investigational Phase 4 68538-85-2, 58-05-9, 73951-54-9 149436 6006
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 Folic Acid Antagonists Phase 4
6 Folate Phase 4
7 Anti-Bacterial Agents Phase 4
8 Vitamin B9 Phase 4
9 Antiprotozoal Agents Phase 4
10 Antiparasitic Agents Phase 4
11 Vitamin B Complex Phase 4
12 Antimalarials Phase 4
13
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
14
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
15
Prednisolone Approved, Vet_approved Phase 2 50-24-8 4894 5755
16
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5 1875
17
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 4159 6741
18
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7 4897
19 Antineoplastic Agents, Hormonal Phase 2
20 Neuroprotective Agents Phase 2
21 Hormones Phase 2
22 Hormone Antagonists Phase 2
23 Antiemetics Phase 2
24 Anti-Inflammatory Agents Phase 2
25 glucocorticoids Phase 2
26
Methylprednisolone Acetate Phase 2 584547
27 Gastrointestinal Agents Phase 2
28 Protective Agents Phase 2
29
Lenograstim Approved, Investigational 135968-09-1
30
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
31
Prednisone Approved, Vet_approved 53-03-2 5865
32
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
33
Clotrimazole Approved, Vet_approved 23593-75-1 2812
34 Antirheumatic Agents
35 Alkylating Agents
36 Antineoplastic Agents, Alkylating
37 Calcineurin Inhibitors
38 Cyclosporins
39 Antifungal Agents
40 Antilymphocyte Serum
41 Immunosuppressive Agents
42 Dermatologic Agents
43 Immunologic Factors

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

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Genetic Tests for Hypersensitivity Vasculitis

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Anatomical Context for Hypersensitivity Vasculitis

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Organs/tissues related to Hypersensitivity Vasculitis:

MalaCards : Skin, Bone, Neutrophil, Bone Marrow, Endothelial, Liver, Colon
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Publications for Hypersensitivity Vasculitis

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Articles related to Hypersensitivity Vasculitis:

(show top 50) (show all 2349)
# Title Authors PMID Year
1
An adult patient with Kabuki syndrome presenting with Henoch-Schönlein purpura complicated with pulmonary hemorrhage. 53 62
19011790 2008
2
Acute hemorrhagic edema of infancy: a case report. 53 62
18328221 2007
3
Henoch-Schonlein purpura with hemorrhagic bullae in children: report of two cases. 53 62
15599471 2004
4
Immunoglobulin A-associated lymphocytic vasculopathy: a clinicopathologic study of eight patients. 53 62
12453297 2002
5
Evolution of immunoglobulin A nephropathy into Henoch-Schönlein purpura in an adult patient. 53 62
7847363 1995
6
Fc gamma receptor activation of neutrophils in cryoglobulin-induced leukocytoclastic vasculitis. 53 62
8318044 1993
7
IgA Vasculitis With Concurrent ANCA-Positivity in the Setting of Bacterial Endocarditis. 62
36413453 2022
8
Typical cutaneous small-vessel vasculitis induced by combined injection of antiphosphatidylserine/prothrombin complex antibody and anti-LAMP-2 antibody in normal rats. 62
35876151 2022
9
Infections or Vaccines Associated with Finkelstein-Seidlmayer Vasculitis: Systematic Review. 62
35553000 2022
10
Atypical Presentation of Leukocytoclastic Vasculitis With Koebner Phenomenon. 62
36413103 2022
11
Cutaneous and Mucosal Conditions Associated With Cocaine Use. 62
36470395 2022
12
Cutaneous manifestations of antineutrophil cytoplasmic antibody-associated vasculitis (AAV): a concise review with emphasis on clinical and histopathologic correlation. 62
35599359 2022
13
Osimertinib-Induced Cutaneous Vasculitis Responsive to Low-Dose Dapsone Without Interruption of Anticancer Therapy: A Case Report and Review of the Literature. 62
36275908 2022
14
Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation. 62
36467823 2022
15
Response to Berry et al's "Cutaneous small-vessel vasculitis following single-dose Janssen Ad26.COV2.S vaccination". 62
35155725 2022
16
Leukocytoclastic vasculitis and COVID-19 vaccination. 62
36162200 2022
17
Systemic disease in leukocytoclastic vasculitis: A focus on direct immunofluorescence findings. 62
36369199 2022
18
Tofacitinib for the treatment of refractory or glucocorticoid-dependent cutaneous leukocytoclastic vasculitis. 62
35988047 2022
19
A cohort study to assess risk of cutaneous small vessel vasculitis among users of different oral anticoagulants. 62
35909259 2022
20
A homozygous truncating mutation of FGL2 is associated with immune dysregulation. 62
36243222 2022
21
Cutaneous Small Vessel Vasculitis: A Practical Guide to Diagnosis and Management. 62
36308673 2022
22
Cutaneous leukocytoclastic vasculitis of the leg, ulcerations, and subungual splinter hemorrhages: rare adverse reactions caused by cabozantinib treatment. 62
36213970 2022
23
Leukocytoclastic Vasculitis and SARS-COV-2 Vaccination. 62
36462800 2022
24
In Urticarial Vasculitis, Long Disease Duration, High Symptom Burden, and High Need for Therapy Are Linked to Low Patient-Reported Quality of Life. 62
35868452 2022
25
Leukocytoclastic vasculitis presenting clinically as bullous pyoderma gangrenosum following leucovorin, fluorouracil and oxaliplatin chemotherapy: a rare case report and literature review. 62
35946554 2022
26
IgA Vasculitis in the Setting of Biologic Therapy for Psoriasis and Recurrent Cutaneous Methicillin-Resistant Staphylococcus aureus Colonization. 62
36446091 2022
27
Amiodarone-induced cutaneous leukocytoclastic vasculitis: a case report and a review of the literature. 62
35654917 2022
28
Hypocomplementemic Urticarial Vasculitis Associated With Hashimoto's Thyroiditis and Hepatitis B Virus Infection: A Case Report. 62
36320953 2022
29
Leukocytoclastic vasculitis mimicking photosensitivity induced by thiamazole. 62
34454743 2022
30
Vasculitis flare after COVID-19: report of two cases in patients with preexistent controlled IgA vasculitis and review of the literature. 62
35691980 2022
31
Superficial (nodular) thrombophlebitis as a heterogeneous entity with distinctive clinico-pathological aspects: Correlation with the underlying conditions. 62
36178454 2022
32
A case of crescentic glomerulonephritis induced by afatinib for lung adenocarcinoma. 62
36180718 2022
33
Torasemide-induced Vascular Purpura in the Course of Eosinophilic Granulomatosis with Polyangiitis. 62
36254546 2022
34
Cutaneous and Mucosal Conditions Associated With Cocaine Use. 62
36115385 2022
35
Trimethoprim-Sulfamethoxazole-Induced Type 2 Cryoglobulinemia and Leukocytoclastic Vasculitis. 62
36074990 2022
36
A Case of Leukocytoclastic Vasculitis Following SARS-COV-2 Vaccination. 62
35690533 2022
37
De novo vasculitis after COVID-19 vaccination. 62
35980076 2022
38
New-onset kidney biopsy-proven IgA vasculitis after receiving mRNA-1273 COVID-19 vaccine: case report. 62
35075622 2022
39
A case of pathologically confirmed streptococcal infection-related IgA vasculitis with associated glomerulonephritis and leukocytoclastic cutaneous vasculitis. 62
35157249 2022
40
Livedoid skin reaction to 2nd dose of mRNA Covid-19 vaccine. 62
36136900 2022
41
Misdiagnosis of neutrophilic erythema of infancy as leukocytoclastic vasculitis: A potential diagnostic pitfall in an infantile inflammatory dermatosis. 62
36000215 2022
42
Sulfasalazine induced leukocytoclastic vasculitis resulting in gangrene. 62
36005861 2022
43
Small-Vessel Vasculitis After ST-Segment Elevation Myocardial Infarction: Clopidogrel or Amiodarone? 62
35935157 2022
44
Leukocytoclastic Vasculitis Following COVID-19 Vaccination: A Case Report. 62
35898243 2022
45
Extensive cutaneous leukocytoclastic vasculitis after Sinopharm vaccine: Case report and review of the literature. 62
35355299 2022
46
Small vessel vasculitis associated with culture-negative infective endocarditis related to a cardiac device: a case report. 62
35935394 2022
47
Platelet count and plateletcrit: Potential haematological biomarkers for livedoid vasculopathy? 62
35635484 2022
48
Use of granulocyte and monocyte adsorption apheresis in dermatology (Review). 62
35837066 2022
49
Cutaneous manifestations following COVID-19 vaccination: A report of 25 cases. 62
35716105 2022
50
Correct approach in urticarial vasculitis made early diagnosis of lupus nephritis possible: a case report. 62
35989318 2022
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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:

# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 13.35 TNF SERPINA3 PRTN3 MPO MEFV IL6
2
Hematopoietic Stem Cells and Lineage-specific Markers 65
11.62 TNF MPO IL6 CD79A
3
Extrafollicular B cell activation by SARS-CoV-2 74
10.95 TNF IL6 ICOSLG CRP CD79A C3
4
Overview of nanoparticle effects 74
10.61 TNF IL6 CRP
5
Cells and molecules involved in local acute inflammatory response 74
Show member pathways
 10.02 TNF IL6
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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 space GO:0005615 9.98 ACE ALB C3 CFHR2 CRP IL6
2 azurophil granule lumen GO:0035578 9.76 SERPINA3 PRTN3 MPO C3
3 extracellular region GO:0005576 9.7 TNF SERPINA3 PRTN3 MPO IL6 FCGR3B

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 10.07 TNF IL6 FCGR3B FCAR CCR6 C3
2 inflammatory response GO:0006954 10.03 C3 CRP IL6 MEFV SERPINA3 TNF
3 acute-phase response GO:0006953 9.8 SERPINA3 IL6 CRP
4 O-glycan processing, core 1 GO:0016267 9.46 C1GALT1C1 C1GALT1
5 response to gold nanoparticle GO:1990268 9.26 TNF MPO
6 negative regulation of lipid storage GO:0010888 9.1 TNF IL6 CRP

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

# Name GO ID Score Top Affiliating Genes
1 glycoprotein-N-acetylgalactosamine 3-beta-galactosyltransferase activity GO:0016263 8.92 C1GALT1C1 C1GALT1
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Sources for Hypersensitivity Vasculitis

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2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 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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