MCID: CNT046
MIFTS: 42

Central Nervous System Vasculitis

Categories: Cardiovascular diseases, Neuronal diseases

Aliases & Classifications for Central Nervous System Vasculitis

MalaCards integrated aliases for Central Nervous System Vasculitis:

Name: Central Nervous System Vasculitis 12 15
Vasculitis, Central Nervous System 71

Classifications:



External Ids:

Disease Ontology 12 DOID:525
MeSH 43 D020293
NCIt 49 C84622
UMLS 71 C0751878

Summaries for Central Nervous System Vasculitis

Disease Ontology : 12 A vasculitis that is characterized by inflammation of blood vessel walls in the brain or spine.

MalaCards based summary : Central Nervous System Vasculitis, also known as vasculitis, central nervous system, is related to purpura and polyarteritis nodosa. An important gene associated with Central Nervous System Vasculitis is SERPINA3 (Serpin Family A Member 3), and among its related pathways/superpathways are Akt Signaling and Cytokine Signaling in Immune system. The drugs Antibodies, Monoclonal and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include brain, skin and t cells, and related phenotypes are hematopoietic system and endocrine/exocrine gland

Related Diseases for Central Nervous System Vasculitis

Diseases related to Central Nervous System Vasculitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 761)
# Related Disease Score Top Affiliating Genes
1 purpura 30.7 TNF IL6 CRP CD40LG
2 polyarteritis nodosa 30.5 PRTN3 MPO IL6 HLA-DRB1 CRP
3 relapsing polychondritis 30.5 IL6 IL17A CRP
4 cryoglobulinemia 30.5 TNF IL6 HLA-DRB1 CD40LG
5 histiocytosis 30.5 TNF SERPINA3 IL17A CCR6
6 meningitis 30.5 TNF IL6 IL17A CRP CD40LG
7 acute disseminated encephalomyelitis 30.5 HLA-DRB1 CD40LG CCR6
8 graft-versus-host disease 30.3 TNF IL6 HLA-DRB1
9 polyradiculoneuropathy 30.3 IL17A HLA-DRB1 CD40LG
10 endophthalmitis 30.3 TNF IL6 CRP CD4
11 anca-associated vasculitis 30.2 PRTN3 MPO HLA-DRB1 CTLA4
12 vascular disease 30.2 TNF MPO IL6 CRP CD40LG CD4
13 langerhans cell histiocytosis 30.2 IL17A CD68 CCR6
14 infective endocarditis 30.2 TNF IL6 IL17A CRP
15 bacterial meningitis 30.2 TNF IL6 IL17A CRP
16 familial mediterranean fever 30.2 TNF IL6 IL17A CRP
17 henoch-schoenlein purpura 30.1 SERPINA3 PRTN3 MPO CRP CD40LG
18 neurosarcoidosis 30.1 TNFRSF1B TNF CRP CD8A CD4
19 mixed connective tissue disease 30.1 TNF IL6 HLA-DRB1 CD40LG
20 nervous system disease 30.1 TNF SERPINA3 IL6 CD4 CCR6
21 polyneuropathy 30.1 TNF IL6 CRP CD40LG
22 facial paralysis 29.9 PRTN3 CRP CD8A CD40LG CD4
23 endocarditis 29.9 TNFRSF1B TNF IL6 IL17A CRP
24 microscopic polyangiitis 29.9 TNF MPO HLA-DRB1 CTLA4
25 cryptococcosis 29.8 TNF IL17A CD8A CD4 CCR6
26 uveitis 29.8 TNFRSF1B TNF IL6 IL17A CTLA4
27 central nervous system disease 29.8 TNF SERPINA3 IL6 IL17A CD4 CCR6
28 interstitial lung disease 29.7 TNF PRTN3 IL6 IL17A CRP CD40LG
29 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 29.7 PTPN22 IL17A CTLA4
30 vasculitis 29.7 TNFRSF1B TNF SERPINA3 PTX3 PRTN3 MPO
31 kawasaki disease 29.7 TNF IL6 IL17A CRP CD40LG CD4
32 proteasome-associated autoinflammatory syndrome 1 29.6 TNFRSF1B TNF MPO IL6 IL17A CRP
33 chickenpox 29.6 TNFRSF1B TNF IL6 CRP CD8A CD40LG
34 leptospirosis 29.5 TNF PTX3 IL6 HLA-DRB1 CRP CD40LG
35 systemic scleroderma 29.5 TNF IL6 IL17A CRP CD8A CD40LG
36 thyroiditis 29.5 TNF PTPN22 IL6 CTLA4
37 acquired immunodeficiency syndrome 29.4 TNFRSF1B TNF IL6 CRP CD40LG CD4
38 syphilis 29.4 IL17A HLA-DRB1 CRP CD8A CD40LG CD4
39 graves' disease 29.4 PTPN22 MPO HLA-DRB1 CTLA4
40 childhood type dermatomyositis 29.2 TNFRSF1B TNF IL6 CD8A CD40LG CD4
41 lupus erythematosus 29.2 TNFRSF1B TNF PTPN22 HLA-DRB1 CTLA4 CD40LG
42 herpes zoster 29.2 TNFRSF1B TNF HLA-DRB1 CRP CD8A CD40LG
43 vitiligo-associated multiple autoimmune disease susceptibility 1 29.2 PTPN22 CTLA4 CD8A CD4 CCR6
44 chorioretinitis 29.2 TNFRSF1B TNF IL6 IL17A CD8A CD40LG
45 bronchopneumonia 29.1 TNF SERPINA3 MPO IL6 CRP CD8A
46 aortitis 29.1 TNFRSF1B TNF PRTN3 MPO IL6 IL17A
47 myocarditis 29.1 TNF IL6 IL17A CTLA4 CD68 CD40LG
48 lung disease 28.9 TNF SERPINA3 PRTN3 MPO IL6 IL17A
49 takayasu arteritis 28.8 TNF PTX3 PTPN22 IL6 HLA-DRB1 CRP
50 crohn's disease 28.8 TNFRSF1B TNF PTPN22 IL6 IL17A HLA-DRB1

Graphical network of the top 20 diseases related to Central Nervous System Vasculitis:



Diseases related to Central Nervous System Vasculitis

Symptoms & Phenotypes for Central Nervous System Vasculitis

MGI Mouse Phenotypes related to Central Nervous System Vasculitis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.22 BLOC1S6 CCR6 CD4 CD40LG CD68 CD8A
2 endocrine/exocrine gland MP:0005379 10.11 CD4 CD40LG CD8A CTLA4 IL17A IL6
3 cardiovascular system MP:0005385 10.1 BLOC1S6 CCR6 CD40LG CRP CTLA4 IL6
4 immune system MP:0005387 10.09 BLOC1S6 CCR6 CD4 CD40LG CD68 CD8A
5 homeostasis/metabolism MP:0005376 10.07 BLOC1S6 CCR6 CD4 CD40LG CRP CTLA4
6 integument MP:0010771 9.61 BLOC1S6 CD4 CD40LG CD8A CTLA4 IL17A
7 respiratory system MP:0005388 9.23 BLOC1S6 CTLA4 IL17A IL6 PTPN22 PTX3

Drugs & Therapeutics for Central Nervous System Vasculitis

Drugs for Central Nervous System Vasculitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antibodies, Monoclonal Phase 1, Phase 2
2 Antibodies Phase 1, Phase 2
3 Immunoglobulins Phase 1, Phase 2
4
Glutamic acid Approved, Nutraceutical 56-86-0 33032

Interventional clinical trials:

(show all 15)
# Name Status NCT ID Phase Drugs
1 A Phase Ib Study of Milatuzumab Administered Subcutaneously in Patients With Active Systemic Lupus Erythematosus (SLE) Unknown status NCT01845740 Phase 1, Phase 2 milatuzumab;Placebo
2 The Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
3 Retrospective Study About 17 Cases of Child With Bacterial Meningitis Coupled With Cerebral Vasculitis Completed NCT03595761
4 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
5 Educational Needs of Patients With Systemic Vasculitis- an International Survey Completed NCT02190929
6 Illness Perception, Fatigue, and Function in Systemic Vasculitis (The VCRC Vasculitis Perception (VIP) Study) Completed NCT02190916
7 Impact of Vasculitis on Employment and Income. An Online Survey of Participants in the VCRC Patient Contact Registry Completed NCT02476292
8 Magnetic Resonance Spectroscopy, Perfusion and Diffusion Tensor Imaging in Neuropsychiatric Lupus Completed NCT00723671
9 Magnetic Resonance Spectroscopy, Perfusion, and Diffusion Tensor Imaging in Neuropsychiatric Lupus Completed NCT00730002
10 Libman-Sacks Endocarditis as a Cause of Neuropsychiatric Systemic Lupus Erythematosus Completed NCT00858884
11 The Vasculitis Pregnancy Registry (V-PREG) Recruiting NCT02593565
12 The Utility of Intracranial Vessel Wall Imaging for Suspected CNS Vasculitis Diagnosis and Assessment of Disease Activity. Recruiting NCT03166319
13 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Recruiting NCT02006134
14 High Resolution Vessel Wall Imaging by Magnetic Resonance Imaging of Intracranial Vasculopathies Recruiting NCT03032809
15 Development of a Novel Glutamate Receptor Ligand for PET Scans in Neuropsychiatric Systemic Lupus Erythematosus Active, not recruiting NCT02456168

Search NIH Clinical Center for Central Nervous System Vasculitis

Genetic Tests for Central Nervous System Vasculitis

Anatomical Context for Central Nervous System Vasculitis

MalaCards organs/tissues related to Central Nervous System Vasculitis:

40
Brain, Skin, T Cells, Lung, B Cells, Bone, Eye

Publications for Central Nervous System Vasculitis

Articles related to Central Nervous System Vasculitis:

(show top 50) (show all 343)
# Title Authors PMID Year
1
Differentiation between neurosarcoidosis and primary central nervous system vasculitis based on demographic, cerebrospinal and imaging features. 61
31928590 2020
2
Central nervous system vasculitis: advances in diagnosis. 61
31652165 2020
3
Expanding the clinical features of autoinflammation and phospholipase Cγ2-associated antibody deficiency and immune dysregulation by description of a novel patient. 61
31465591 2019
4
Vessel Wall Imaging of Cerebrovascular Disorders. 61
31728661 2019
5
Development of Central Nervous System Vasculitis in a Patient with Waldenstrom Macroglobulinemia: A Rare Presentation with Poor Prognosis. 61
31824806 2019
6
Central nervous system vasculitis from Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorder in children: A case report. 61
31208818 2019
7
The diagnosis of primary central nervous system vasculitis. 61
31649101 2019
8
A Case of Primary Central Nervous System Vasculitis That Worsened Despite Early Corticosteroid Therapy. 61
31686451 2019
9
Concordance of angiography and cerebral biopsy results for suspected primary central nervous system vasculitis: A multi-center retrospective review. 61
31421586 2019
10
Elevated cerebrospinal fluid levels of total protein in patients with secondary central nervous system vasculitis and giant cell arteritis. 61
31599690 2019
11
Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis. 61
31451471 2019
12
Clinical Manifestations and Mechanisms of Autoimmune Disease-Related Multiple Cerebral Infarcts. 61
31062611 2019
13
Susceptibility-weighted imaging in the differential diagnosis of autoimmune central nervous system vasculitis and multiple sclerosis. 61
31158805 2019
14
Improvement of Central Nervous System Vasculitis in a Patient with Chronic Hepatitis C Virus Infection after Treatment with an Interferon-Free Regimen. 61
31328142 2019
15
A case of central nervous system vasculitis presenting as a mass-like lesion. 61
30610477 2019
16
Utility of diagnostic cerebral angiography in the management of suspected central nervous system vasculitis. 61
30952556 2019
17
Relapsing Primary Central Nervous System Vasculitis Treated With Rituximab. 61
31166213 2019
18
A systemic lupus erythematosus patient with thunderclap headache: reversible cerebral vasoconstriction syndrome. 61
31042127 2019
19
Bilateral intracerebral hemorrhage as a presentation of central nervous system vasculitis in a patient with dermatomyositis. 61
31211252 2019
20
Cognitive outcomes of childhood primary CNS vasculitis. 61
30762375 2019
21
X-Linked Lymphoproliferative Syndrome Presenting as Adult-Onset Multi-Infarct Dementia. 61
30990878 2019
22
Central Nervous System Vasculitis Due to Substance Abuse. 61
30952417 2019
23
Central Nervous System Vasculitis due to Infection. 61
30952418 2019
24
A Long Interval from a Spinal Cord Lesion to a Subsequent Brain Lesion in Primary Central Nervous System Vasculitis. 61
30449796 2019
25
MOG antibody disease manifesting as progressive cognitive deterioration and behavioral changes with primary central nervous system vasculitis. 61
30738279 2019
26
Eleven Themes in the History of Systemic and Nervous System Vasculitides. 61
30952403 2019
27
The Blood-Brain Barrier: Implications for Vasculitis. 61
30952407 2019
28
Anti-neutrophil cytoplasmic antibody associated central nervous system vasculitis with brain and spinal cord subarachnoid hemorrhage: A rare case report and review of the literature. 61
30594448 2019
29
Rituximab therapy for primary central nervous system vasculitis: A 6 patient experience and review of the literature. 61
30743080 2019
30
Study on the Clinical, Imaging, and Pathological Characteristics of 18 Cases with Primary Central Nervous System Vasculitis. 61
30635219 2019
31
Hospital Onset Varicella Central Nervous System Vasculitis in a Patient with HIV Infection. 61
30715900 2019
32
Primary central nervous system vasculitis mimicking brain tumor: Comprehensive analysis of 13 cases from a single institutional cohort of 191 cases. 61
30528173 2019
33
Recurrent strokes, central nervous system vasculitis, and acquired protein S deficiency secondary to varicella zoster in a child with AIDS. 61
30478796 2019
34
Fatal Central Nervous System Lymphocytic Vasculitis after Treatment for Burkitt Lymphoma in a Patient with a SH2D1A Mutation. 61
30138256 2019
35
Endothelins in inflammatory neurological diseases. 61
30291906 2019
36
Langerhans Cell Histiocytosis Causing Central Nervous System Vasculitis Leading to Stroke in a Two-year-old Boy: A Case Report. 61
30937249 2019
37
[Primary central nervous system vasculitis]. 61
31626179 2019
38
[Toxoplasmosis-associated central nervous system vasculitis accompanied by multiple cerebral hemorrhages developing subsequent to cord blood transplantation]. 61
30842378 2019
39
Primary central nervous system vasculitis in India - need for a multicenter prospective cohort study. 61
30860107 2019
40
Vessel wall enhancement on MRI in the diagnosis of primary central nervous system vasculitis. 61
30102571 2018
41
High-resolution Intracranial Vessel Wall Imaging in Monitoring Treatment Response in Primary CNS Angiitis. 61
30379741 2018
42
FET PET in Primary Central Nervous System Vasculitis. 61
30080188 2018
43
Treatment and Long-Term Outcomes of Primary Central Nervous System Vasculitis. 61
29986936 2018
44
Central Nervous System Vasculitis for Cryptococcosis in an Immunocompetent Patient. 61
30200288 2018
45
The first case of bacillus Calmette-Guérin-induced small-vessel central nervous system vasculitis. 61
29740727 2018
46
Vascular wall imaging in reversible cerebral vasoconstriction syndrome - a 3-T contrast-enhanced MRI study. 61
30167985 2018
47
Cerebral Infarction and Cortical Subarachnoid Hemorrhage Preceded Vascular Contraction in Reversible Cerebral Vasoconstriction Syndrome Patient with Hidden Breast Cancer. 61
30258266 2018
48
A case of craniopharyngioma presenting as rapidly progressive dementia. 61
30405265 2018
49
Pyogenic Meningitis Complicated with Extensive Central Nervous System Vasculitis and Moyamoya Vasculopathy. 61
30271471 2018
50
Isolated Cervical Cord Involvement in Primary Central Nervous System Vasculitis. 61
29961677 2018

Variations for Central Nervous System Vasculitis

Expression for Central Nervous System Vasculitis

Search GEO for disease gene expression data for Central Nervous System Vasculitis.

Pathways for Central Nervous System Vasculitis

Pathways related to Central Nervous System Vasculitis according to GeneCards Suite gene sharing:

(show all 38)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.37 TNFRSF1B TNFRSF10A TNF IL6 IL17A CD40LG
2
Show member pathways
13.31 TNFRSF1B TNF PRTN3 IL6 IL17A HLA-DRB1
3
Show member pathways
13.21 TNFRSF1B TNFRSF10A TNF PTPN22 IL6 IL17A
4
Show member pathways
13.15 TNFRSF1B TNF SERPINA3 PTX3 PTPN22 PRTN3
5
Show member pathways
12.66 TNF IL6 IL17A HLA-DRB1 CTLA4 CD8A
6
Show member pathways
12.6 TNFRSF1B TNF HLA-DRB1 CD40LG CD4
7
Show member pathways
12.56 TNF IL6 IL17A HLA-DRB1 CD4
8
Show member pathways
12.55 TNF MPO IL6 IL17A CD4
9
Show member pathways
12.49 TNF IL6 IL17A CTLA4 CD8A CD40LG
10
Show member pathways
12.36 HLA-DRB1 CTLA4 CD40LG CD4
11
Show member pathways
12.31 TNFRSF1B TNFRSF10A TNF IL6 CCR6
12 12.3 TNF IL6 HLA-DRB1 CD4
13
Show member pathways
12.3 TNF SERPINA3 MPO IL6 CRP
14 12.22 TNFRSF1B TNFRSF10A TNF CD40LG
15 12.08 HLA-DRB1 CTLA4 CD8A CD40LG CD4
16 12.07 TNFRSF1B TNF PTPN22 MPO IL17A CTLA4
17
Show member pathways
12.02 TNF IL6 IL17A CD4
18 11.94 TNFRSF1B TNF IL6 IL17A
19 11.87 TNF IL6 HLA-DRB1 CD8A CD4
20 11.82 TNF IL6 IL17A HLA-DRB1 CTLA4
21 11.74 PRTN3 MPO CD4
22
Show member pathways
11.73 TNF CTLA4 CD40LG
23 11.72 TNF IL6 CD8A CD4
24
Show member pathways
11.71 TNFRSF1B TNF CD40LG
25 11.7 TNF MPO IL6 CD8A CD68 CD4
26 11.68 TNF IL6 IL17A CD68
27 11.67 TNF IL17A CD8A CD4 CCR6
28
Show member pathways
11.63 TNF IL6 CD40LG
29 11.63 TNF PTX3 IL6
30 11.52 TNF IL6 CD40LG
31 11.51 PTPN22 HLA-DRB1 CTLA4 CD8A CD4
32 11.5 TNFRSF1B TNF IL6
33 11.49 TNF MPO IL6
34 11.37 CD8A CD40LG CD4
35 11.27 TNF IL6 IL17A
36 11.2 TNF IL6 HLA-DRB1 CD4
37 11.01 TNF IL6 CRP
38 10.92 TNF MPO CD68

GO Terms for Central Nervous System Vasculitis

Cellular components related to Central Nervous System Vasculitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.81 TNF SERPINA3 PTX3 PRTN3 MPO IL6
2 cell surface GO:0009986 9.73 TNFRSF10A TNF HLA-DRB1 CD40LG CD4 CCR6
3 extracellular region GO:0005576 9.7 TNFRSF1B TNF SERPINA3 PTX3 PRTN3 MPO
4 membrane raft GO:0045121 9.62 TNFRSF1B TNF PRTN3 CD4
5 external side of plasma membrane GO:0009897 9.23 TNF IL17A HLA-DRB1 CTLA4 CD8A CD40LG

Biological processes related to Central Nervous System Vasculitis according to GeneCards Suite gene sharing:

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 cell surface receptor signaling pathway GO:0007166 9.86 TNFRSF1B TNFRSF10A CD8A CD4
2 cellular response to lipopolysaccharide GO:0071222 9.8 TNFRSF1B TNF IL6 CD68
3 neutrophil degranulation GO:0043312 9.8 TNFRSF1B SERPINA3 PTX3 PRTN3 MPO CD68
4 T cell receptor signaling pathway GO:0050852 9.78 PTPN22 HLA-DRB1 CTLA4 CD4
5 cytokine-mediated signaling pathway GO:0019221 9.73 TNFRSF1B TNF PRTN3 IL6 IL17A CD4
6 positive regulation of T cell proliferation GO:0042102 9.7 IL6 CD40LG CD4
7 humoral immune response GO:0006959 9.69 TNF IL6 CCR6
8 extrinsic apoptotic signaling pathway GO:0097191 9.61 TNFRSF1B TNFRSF10A TNF
9 inflammatory response GO:0006954 9.56 TNFRSF1B TNF SERPINA3 PTX3 IL6 IL17A
10 regulation of neuroinflammatory response GO:0150077 9.55 TNFRSF1B IL6
11 opsonization GO:0008228 9.54 PTX3 CRP
12 acute-phase response GO:0006953 9.54 SERPINA3 IL6 CRP
13 regulation of immunoglobulin secretion GO:0051023 9.46 TNF CD40LG
14 response to yeast GO:0001878 9.4 PTX3 MPO
15 negative regulation of lipid storage GO:0010888 9.33 TNF IL6 CRP
16 immune response GO:0006955 9.32 TNFRSF1B TNF IL6 IL17A HLA-DRB1 CTLA4

Molecular functions related to Central Nervous System Vasculitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.26 TNF IL6 IL17A CD40LG
2 complement component C1q binding GO:0001849 8.62 PTX3 CRP

Sources for Central Nervous System Vasculitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....