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PAN
MCID: PLY017
MIFTS: 60

Polyarteritis Nodosa (PAN)

Categories: Bone diseases, Cardiovascular diseases, Nephrological diseases, Neuronal diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Polyarteritis Nodosa

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MalaCards integrated aliases for Polyarteritis Nodosa:

Name: Polyarteritis Nodosa 12 73 20 58 36 29 54 6 44 15 17 70 32
Periarteritis 20 70
Polyarteritis 20 70
Pan 20 58
Kussmaul-Maier Disease 58
Periarteritis Nodosa 58

Characteristics:

Orphanet epidemiological data:

58
polyarteritis nodosa
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

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


External Ids:

Disease Ontology 12 DOID:9810
KEGG 36 H01382
ICD9CM 34 446.0
MeSH 44 D010488
NCIt 50 C26847
SNOMED-CT 67 195347004
ICD10 32 M30.0 M30.1
MESH via Orphanet 45 D010488
ICD10 via Orphanet 33 M30.0
UMLS via Orphanet 71 C0031036
Orphanet 58 ORPHA767
UMLS 70 C0031036 C0264988 C0264992
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Summaries for Polyarteritis Nodosa

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GARD : 20 Polyarteritis nodosa (PAN) is a blood vessel disease characterized by inflammation of small and medium-sized arteries ( vasculitis ), preventing them from bringing oxygen and food to organs. Most cases occur in the 4th or 5th decade of life, although it can occur at any age. PAN most commonly affects vessels related to the skin, joints, peripheral nerves, gastrointestinal tract, heart, eyes, and kidneys. Symptoms are caused by damage to affected organs and may include fever, fatigue, weakness, loss of appetite, weight loss, muscle and joint aches, rashes, numbness, and abdominal pain. The underlying cause of PAN is unknown. Treatment involves medicines to suppress inflammation and the immune system, including steroids.

MalaCards based summary : Polyarteritis Nodosa, also known as periarteritis, is related to cutaneous polyarteritis nodosa and microscopic polyangiitis. An important gene associated with Polyarteritis Nodosa is ADA2 (Adenosine Deaminase 2), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Methylprednisolone and Prednisolone have been mentioned in the context of this disorder. Affiliated tissues include neutrophil, lung and endothelial, and related phenotypes are fever and arthralgia

Disease Ontology : 12 A vasculitis that is characterized by necrotizing inflammation of medium or small arteries.

KEGG : 36 Polyarteritis nodosa is a systemic necrotizing vasculitis that affects medium and small muscular arteries. The ensuing tissue ischemia can affect any organ, including the skin, musculoskeletal system, kidneys, gastrointestinal tract, and the cardiovascular and nervous systems. In most cases, onset of the disease occurred during childhood. Polyarteritis nodosa is most often primary. Recessive mutations in the adenosine deaminase 2 (ADA2)-encoding gene CECR1 have been found from patients of this disease.

Wikipedia : 73 Polyarteritis nodosa (PAN), is a systemic necrotizing inflammation of blood vessels (vasculitis)... more...

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Related Diseases for Polyarteritis Nodosa

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Diseases in the Polyarteritis Nodosa family:

Systemic Polyarteritis Nodosa Secondary Polyarteritis Nodosa
Primary Polyarteritis Nodosa

Diseases related to Polyarteritis Nodosa via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 809)
# Related Disease Score Top Affiliating Genes
1 cutaneous polyarteritis nodosa 33.3 SMAD3 IFNA2
2 microscopic polyangiitis 31.7 MPO HLA-DRB1
3 churg-strauss syndrome 31.5 PRTN3 MPO HLA-DRB1
4 familial mediterranean fever 31.4 MEFV IL6 CRP
5 purpura 31.3 MEFV IL6 CRP APOH
6 exanthem 31.2 PRTN3 MEFV IL6 GPT CRP
7 mononeuritis multiplex 31.2 PRTN3 MPO CRP ADA2
8 hepatitis b 31.1 IL6 IFNA2 HLA-DRB1 GPT
9 glomerulonephritis 31.1 VCAM1 PRTN3 MPO ICAM1
10 intestinal perforation 31.1 IL6 GPT CRP
11 kawasaki disease 31.0 SELL SELE IL6 GPT CRP
12 mononeuropathy 30.9 PRTN3 MPO CRP
13 thrombocytosis 30.9 SELP IL6 CRP
14 branch retinal artery occlusion 30.9 PRTN3 CRP APOH
15 granulomatous angiitis 30.9 PRTN3 CRP
16 retinal artery occlusion 30.9 IL6 CRP APOH
17 anca-associated vasculitis 30.8 PRTN3 MPO
18 cryoglobulinemia 30.8 IL6 IFNA2 HLA-DRB1 GPT APOH
19 brucellosis 30.8 SELL MPO MEFV IL6 CRP
20 pyelonephritis 30.8 IL6 GPT CRP
21 neuritis 30.7 VCAM1 PECAM1 IL6 HLA-DRB1
22 livedoid vasculitis 30.7 APOH ADA2
23 adult-onset still's disease 30.7 MEFV IL6 CRP
24 connective tissue disease 30.7 SMAD3 IL6 HLA-DRB1 CRP APOH
25 pericarditis 30.7 MEFV IL6 CRP
26 antiphospholipid syndrome 30.7 VCAM1 SELP SELE CRP APOH
27 hepatic infarction 30.7 GPT APOH
28 rapidly progressive glomerulonephritis 30.7 PRTN3 MPO ICAM1 CRP
29 disseminated intravascular coagulation 30.7 IL6 GPT CRP
30 rheumatic fever 30.6 IL6 ICAM1 HLA-DRB1
31 systemic scleroderma 30.6 SELP SELE IL6 CRP
32 acalculous cholecystitis 30.6 GPT CRP
33 appendicitis 30.6 MPO IL6 CRP
34 psoriatic arthritis 30.6 SELE IL6 HLA-DRB1 CRP
35 granulomatosis with polyangiitis 30.6 SELE PRTN3 MPO CRP ADA2
36 viral hepatitis 30.6 IL6 IFNA2 HLA-DRB1 GPT CRP
37 pyoderma gangrenosum 30.6 MEFV IFNA2 APOH
38 amyloidosis aa 30.6 MEFV CRP
39 intestinal obstruction 30.5 IL6 GPT CRP
40 peripheral vascular disease 30.5 VCAM1 SELP SELE IL6 CRP
41 mixed connective tissue disease 30.5 IL6 HLA-DRB1 APOH
42 rheumatic heart disease 30.5 VCAM1 IL6 HLA-DRB1
43 kidney disease 30.5 VCAM1 PRTN3 PECAM1 MEFV IL6 CRP
44 posterior scleritis 30.5 PRTN3 CRP
45 pyoderma 30.5 MEFV IFNA2 CRP APOH
46 acute pancreatitis 30.4 MPO IL6 GPT CRP
47 acute myocardial infarction 30.4 SELP SELE PECAM1 IL6 CRP
48 temporal arteritis 30.4 PRTN3 MPO IL6 HLA-DRB1 CRP APOH
49 vasculitis 30.4 VCAM1 SELP SELL SELE PRTN3 MPO
50 crescentic glomerulonephritis 30.4 VCAM1 SELE PRTN3 MPO ICAM1

Graphical network of the top 20 diseases related to Polyarteritis Nodosa:



Diseases related to Polyarteritis Nodosa
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Symptoms & Phenotypes for Polyarteritis Nodosa

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Human phenotypes related to Polyarteritis Nodosa:

58 31 (show all 27)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
2 arthralgia 58 31 frequent (33%) Frequent (79-30%) HP:0002829
3 myalgia 58 31 frequent (33%) Frequent (79-30%) HP:0003326
4 abnormality of the kidney 58 31 frequent (33%) Frequent (79-30%) HP:0000077
5 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
6 elevated c-reactive protein level 58 31 frequent (33%) Frequent (79-30%) HP:0011227
7 polyneuritis 58 31 frequent (33%) Frequent (79-30%) HP:0031003
8 hypertension 58 31 occasional (7.5%) Occasional (29-5%) HP:0000822
9 skin ulcer 58 31 occasional (7.5%) Occasional (29-5%) HP:0200042
10 subcutaneous nodule 58 31 occasional (7.5%) Occasional (29-5%) HP:0001482
11 abdominal pain 58 31 occasional (7.5%) Occasional (29-5%) HP:0002027
12 pericarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001701
13 erythema 58 31 occasional (7.5%) Occasional (29-5%) HP:0010783
14 abnormality of the gastrointestinal tract 58 31 occasional (7.5%) Occasional (29-5%) HP:0011024
15 cutis marmorata 58 31 occasional (7.5%) Occasional (29-5%) HP:0000965
16 raynaud phenomenon 58 31 occasional (7.5%) Occasional (29-5%) HP:0030880
17 sensory axonal neuropathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0003390
18 morphological central nervous system abnormality 31 occasional (7.5%) HP:0002011
19 abnormality of the eye 58 31 very rare (1%) Very rare (<4-1%) HP:0000478
20 cardiomyopathy 58 31 very rare (1%) Very rare (<4-1%) HP:0001638
21 pleuritis 58 31 very rare (1%) Very rare (<4-1%) HP:0002102
22 peripheral neuropathy 58 Frequent (79-30%)
23 abnormal lung morphology 58 Occasional (29-5%)
24 abnormality of the nervous system 58 Occasional (29-5%)
25 abnormality of cardiovascular system morphology 58 Occasional (29-5%)
26 morphological abnormality of the central nervous system 58 Occasional (29-5%)
27 abnormality of skin morphology 58 Very frequent (99-80%)

GenomeRNAi Phenotypes related to Polyarteritis Nodosa according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability ratio GR00368-A 9.28 APOH HLA-DRB1 IFNA2 MEFV MPO MSN

MGI Mouse Phenotypes related to Polyarteritis Nodosa:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.06 CRP FGF2 ICAM1 IL6 MPO PECAM1
2 cellular MP:0005384 10.03 FGF2 ICAM1 IL6 MEFV MSN PECAM1
3 hematopoietic system MP:0005397 10 FGF2 ICAM1 ICAM2 IL6 MEFV MPO
4 homeostasis/metabolism MP:0005376 9.8 APOH CRP FGF2 ICAM1 IL6 MEFV
5 immune system MP:0005387 9.47 CRP ICAM1 ICAM2 IL6 MEFV MPO
Sources

Drugs & Therapeutics for Polyarteritis Nodosa

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Drugs for Polyarteritis Nodosa (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 62)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
2
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
3
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
4
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
5
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
6
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
7
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
8
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
9
Mycophenolic acid Approved Phase 4 24280-93-1 446541
10
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
11 Neuroprotective Agents Phase 4
12 Alkylating Agents Phase 4
13 Gastrointestinal Agents Phase 4
14 Methylprednisolone Acetate Phase 4
15 glucocorticoids Phase 4
16 Hormone Antagonists Phase 4
17 Dermatologic Agents Phase 4
18 Antineoplastic Agents, Hormonal Phase 4
19 Hormones Phase 4
20 Antiemetics Phase 4
21 Anti-Inflammatory Agents Phase 4
22 Protective Agents Phase 4
23 Antibiotics, Antitubercular Phase 4
24 Antitubercular Agents Phase 4
25
Lamivudine Approved, Investigational Phase 2 134678-17-4 60825
26
Azathioprine Approved Phase 2 446-86-6 2265
27
Levoleucovorin Approved, Investigational Phase 2 68538-85-2 149436
28
Ethanol Approved Phase 2 64-17-5 702
29
Naltrexone Approved, Investigational, Vet_approved Phase 2 16590-41-3 5360515
30
Colchicine Approved Phase 2 64-86-8 6167 2833
31
Dapsone Approved, Investigational Phase 2 80-08-0 2955
32
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
33 Reverse Transcriptase Inhibitors Phase 2
34 Anti-Retroviral Agents Phase 2
35 Antiviral Agents Phase 2
36 Anti-HIV Agents Phase 2
37 Immunosuppressive Agents Phase 2
38 Immunologic Factors Phase 2
39 Antirheumatic Agents Phase 2
40 Anti-Infective Agents Phase 2
41 Antimetabolites Phase 2
42 Vitamin B9 Phase 2
43 Folic Acid Antagonists Phase 2
44 Anti-Bacterial Agents Phase 2
45 Vitamin B Complex Phase 2
46 Folate Phase 2
47 Narcotics Phase 2
48 Narcotic Antagonists Phase 2
49 Tubulin Modulators Phase 2
50 Antimitotic Agents Phase 2

Interventional clinical trials:

(show all 22)
# Name Status NCT ID Phase Drugs
1 CHUSPAN PAN BP Treatment of Polyarteritis Nodosa and Microscopic Polyangiitis Without Poor-Prognosis Factors a Prospective Randomized Study in 125 Patients Unknown status NCT00400075 Phase 4 azathioprine;cyclophosphamide
2 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Comparison of Two Strategies Combining Steroids With or Without Immunosuppressants Completed NCT00307671 Phase 4 prednisone, methylprednisolone,cyclophosphamides;Cyclophosphamide, Azathioprine,prednisone,methylprednisolone;Mycophenolate mofetil,methotrexate
3 Evaluation of a New Treatment Strategy for Patients With Microscopic Polyangiitis, Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome) Without Poor Prognosis Factors Completed NCT00647166 Phase 3 corticosteroid and azathioprine;corticosteroid and placebo
4 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
5 Lamivudine for Chronic Hepatitis B Completed NCT00001457 Phase 2 Lamivudine
6 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
7 A Randomized Multicenter Study for Isolated Skin Vasculitis Recruiting NCT02939573 Phase 2 Colchicine;Dapsone;Azathioprine
8 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055 anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone
9 ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis Unknown status NCT01066208
10 Impact of Vasculitis on Employment and Income. An Online Survey of Participants in the VCRC Patient Contact Registry Completed NCT02476292
11 Educational Needs of Patients With Systemic Vasculitis- an International Survey Completed NCT02190929
12 VCRC Validation of Patient-Reported Diagnostic Data Completed NCT02190942
13 The Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
14 Illness Perception, Fatigue, and Function in Systemic Vasculitis (The VCRC Vasculitis Perception (VIP) Study) Completed NCT02190916
15 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
16 VCRC Tissue Biorepository Collection Protocol Recruiting NCT02967068
17 Studies of the Natural History, Pathogenesis, and Outcome of Idiopathic Systemic Vasculitis Recruiting NCT02257866
18 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
19 The Vasculitis Pregnancy Registry (V-PREG) Recruiting NCT02593565
20 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Recruiting NCT02006134
21 Longitudinal Protocol of Polyarteritis Nodosa Active, not recruiting NCT00315406
22 VCRC Genetic Repository One-Time DNA Protocol Active, not recruiting NCT01241305

Search NIH Clinical Center for Polyarteritis Nodosa

Cochrane evidence based reviews: polyarteritis nodosa

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Genetic Tests for Polyarteritis Nodosa

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Genetic tests related to Polyarteritis Nodosa:

# Genetic test Affiliating Genes
1 Polyarteritis Nodosa 29
Sources

Anatomical Context for Polyarteritis Nodosa

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MalaCards organs/tissues related to Polyarteritis Nodosa:

40
Neutrophil, Lung, Endothelial, Eye, Lymph Node, Kidney, Small Intestine
Sources

Publications for Polyarteritis Nodosa

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Articles related to Polyarteritis Nodosa:

(show top 50) (show all 3510)
# Title Authors PMID Year
1
Mutations/polymorphisms in a monogenetic autoinflammatory disease may be susceptibility markers for certain rheumatic diseases: lessons from the bedside for the benchside. 54 61
19796529 2009
2
Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa. 61 54
18035151 2007
3
Cytomegalovirus infection in systemic necrotizing vasculitis: causative agent or opportunistic infection? 61 54
11149660 2000
4
Clinical, laboratory and molecular characteristics of children with Familial Mediterranean Fever-associated vasculitis. 54 61
10709887 2000
5
[A reversible posterior leukoencephalopathy syndrome in a patient with classical polyarteritis nodosa]. 54 61
9146079 1997
6
Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa. 54 61
7754755 1995
7
[Autoantibodies associated with vasculitis]. 61 54
7933581 1994
8
Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges. 54 61
7912504 1994
9
Elevations of neutrophil proteinase 3 in serum of patients with Wegener's granulomatosis and polyarteritis nodosa. 54 61
7907475 1994
10
Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. 54 61
1466365 1992
11
[Antineutrophil cytoplasmic antibodies--ANCA]. 54 61
1464072 1992
12
Polyarteritis nodosa with perirenal hematoma due to the rupture of a renal artery aneurysm. 61
33175365 2021
13
Bacterial-Infection-Associated Polyarteritis Nodosa Presenting as Acute, Rapidly Progressive Multiple Hepatic Artery Aneurysms. 61
33084540 2021
14
Biologic drugs in the treatment of polyarteritis nodosa and deficit of adenosine deaminase 2: A narrative review. 61
33609794 2021
15
Mycophenolate mofetil versus cyclophosphamide for remission induction in childhood polyarteritis nodosa: An open label, randomised, Bayesian, non-inferiority trial. 61
33760371 2021
16
Expanding spectrum of DADA2: a review of phenotypes, genetics, pathogenesis and treatment. 61
33791889 2021
17
Polyarteritis Nodosa and Membranous Glomerulonephritis: Two Simultaneous Extrahepatic Manifestations of Hepatitis B. 61
29965852 2021
18
Clinical features and outcomes of childhood polyarteritis nodosa: A single referral center experience. 61
33615972 2021
19
The Temporal Relationships and Associations between Cutaneous Manifestations and Inflammatory Bowel Disease: A Nationwide Population-Based Cohort Study. 61
33810197 2021
20
Disseminated Tuberculosis and Saccular Aneurysm Findings on FDG PET/CT in a Patient With Polyarteritis Nodosa. 61
31764382 2021
21
Sequence-Based Screening of Patients With Idiopathic Polyarteritis Nodosa, Granulomatosis With Polyangiitis, and Microscopic Polyangiitis for Deleterious Genetic Variants in ADA2. 61
33021335 2021
22
Pulmonary Involvement in Primary Systemic Vasculitides. 61
33788906 2021
23
Livedo Racemosa: Clinical, Laboratory, and Histopathological Findings in 33 Patients. 61
31996060 2021
24
Paraneoplastic polyarteritis nodosa in a patient with cutaneous T-cell lymphoma. 61
33636144 2021
25
Isolated intestinal polyarteritis nodosa in an elderly patient. 61
33664043 2021
26
[Use of new diagnostic criteria for reclassification of polyarteritis nodosa]. 61
33663173 2021
27
Successful bilateral popliteal-plantar bypasses for polyarteritis nodosa induced ischemia. 61
33718688 2021
28
Characteristics and risk factors for poor outcome in patients with systemic vasculitis involving the gastrointestinal tract. 61
33711774 2021
29
18F-Fluorodeoxyglucose positron emission tomography computed tomography detection of single organ vasculitis of the breast: A case report. 61
33761723 2021
30
Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa. 61
33569653 2021
31
Insidious coronary artery disease in a young patient with polyarteritis nodosa: a case report and literature review. 61
33639843 2021
32
Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis of Test Accuracy and Benefits and Harms of Common Treatments. 61
33512787 2021
33
Burn Center Management of Severe Necrotic Cutaneous Polyarteritis Nodosa in a Patient With a History of Thymoma. 61
33728150 2021
34
Polyarteritis Nodosa: A Systematic Review of Test Accuracy and Benefits and Harms of Common Treatments. 61
33512781 2021
35
Detailed analysis of Japanese patients with adenosine deaminase 2 deficiency reveals characteristic elevation of type II interferon signature and STAT1 hyperactivation. 61
33529688 2021
36
Medium- and Large-Vessel Vasculitis. 61
33464968 2021
37
Inflammatory comments in coronary artery disease: when to suspect polyarteritis nodosa or other primary systemic vasculitis. 61
33453354 2021
38
Human hepatitis viruses-associated cutaneous and systemic vasculitis. 61
33505148 2021
39
The same mutation in a family with adenosine deaminase 2 deficiency. 61
31541281 2021
40
Coronary artery vasculitis: a review of current literature. 61
33407141 2021
41
Vitamin D Levels in Patients With Small and Medium Vessel Vasculitis. 61
33454206 2021
42
Polyarteritis nodosa: resolution of radiological changes. 61
33280000 2020
43
Characteristics and outcomes of coronary artery involvement in polyarteritis nodosa. 61
33310144 2020
44
Group A Streptococcus Infection-Induced Polyarteritis Nodosa in an Adult Patient Complicated With Diffuse Fasciitis. 61
31693651 2020
45
Somatic Mutations in UBA1 and Severe Adult-Onset Autoinflammatory Disease. 61
33108101 2020
46
French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides). 61
33372616 2020
47
Peripheral neuropathy in systemic vasculitis and other autoimmune diseases - a report of five cases emphasizing the importance of etiologic characterization. 61
32995578 2020
48
Two cases of ADA2 deficiency presenting as childhood polyarteritis nodosa: novel ADA2 variant, atypical CNS manifestations, and literature review. 61
32535845 2020
49
Role of therapeutic plasma exchanges in systemic vasculitis. 61
33172734 2020
50
Cutaneous polyarteritis nodosa with clinical features of pyoderma gangrenosum. 61
33294567 2020
Sources

Variations for Polyarteritis Nodosa

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ClinVar genetic disease variations for Polyarteritis Nodosa:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 ADA2 NM_001282225.2(ADA2):c.-46-108_542+142del Deletion Likely pathogenic 973523 GRCh37: 22:17687819-17690721
GRCh38: 22:17206929-17209831
Sources

Expression for Polyarteritis Nodosa

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Search GEO for disease gene expression data for Polyarteritis Nodosa.
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Pathways for Polyarteritis Nodosa

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Pathways related to Polyarteritis Nodosa according to GeneCards Suite gene sharing:

(show all 27)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 65
Show member pathways
 13.81 VCAM1 SELL PRTN3 PECAM1 MPO MEFV
2
Cytokine Signaling in Immune system 65
Show member pathways
 13.28 VCAM1 PRTN3 IL6 IFNA2 ICAM1 HLA-DRB1
3
Response to elevated platelet cytosolic Ca2+ 65
Show member pathways
 13.21 SELP SELL SELE PRTN3 PECAM1 IFNA2
4
Toll-like Receptor Signaling Pathway 36 1
Show member pathways
 13.08 VCAM1 SMAD3 SELP SELE IL6 IFNA2
5
Measles 36
Show member pathways
 12.74 MSN IL6 IFNA2 ICAM1 HLA-DRB1
6
Degradation of the extracellular matrix 65
Show member pathways
 12.65 VCAM1 PECAM1 ICAM2 ICAM1 FGF2
7
Human T-cell leukemia virus 1 infection 36
12.25 SMAD3 IL6 ICAM1 HLA-DRB1
8
Folate Metabolism 1
Show member pathways
 12.24 MPO IL6 ICAM1 CRP
9
Cell surface interactions at the vascular wall 65
Show member pathways
 12.13 SELP SELL SELE PECAM1
10
ECM-receptor interaction 36
Show member pathways
 12.05 VCAM1 PECAM1 ICAM2 ICAM1
11
Fluid shear stress and atherosclerosis 36
11.97 VCAM1 SELE PECAM1 ICAM1
12
Immunoregulatory interactions between a Lymphoid and a non-Lymphoid cell 65
11.94 VCAM1 SELL ICAM2 ICAM1
13
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 64
11.86 SELL MPO IL6 IFNA2
14
Interleukin-4 and 13 signaling 65
11.84 VCAM1 IL6 ICAM1 FGF2
15
TNF signaling pathway 36
11.81 VCAM1 SELE IL6 ICAM1
16
Glucocorticoid receptor regulatory network 1
11.72 SELE IL6 ICAM1
17
Dendritic Cells Developmental Lineage Pathway 64
11.66 SELL IL6 IFNA2
18
AGE-RAGE signaling pathway in diabetic complications 36
11.65 VCAM1 SMAD3 SELE IL6 ICAM1
19
IgA-Producing B Cells in the Intestine 64
11.58 VCAM1 IL6 ICAM1
20
Photodynamic therapy-induced NF-kB survival signaling 1
Show member pathways
 11.5 VCAM1 SELE IL6 ICAM1
21
Thromboxane A2 receptor signaling 1
11.4 VCAM1 SELE ICAM1
22
Cell adhesion molecules 36
11.4 VCAM1 SELP SELL SELE PECAM1 ICAM2
23
Immune response_Role of integrins in NK cells cytotoxicity 23
11.37 VCAM1 ICAM2 ICAM1
24
amb2 Integrin signaling 1
11.23 SELP MPO IL6 ICAM2 ICAM1
25
African trypanosomiasis 36
11.19 VCAM1 SELE IL6 ICAM1
26
Malaria 36
11.13 VCAM1 SELP SELE PECAM1 IL6 ICAM1
27
LDL Oxidation in Atherogenesis 63
10.81 VCAM1 MPO ICAM1
Sources

GO Terms for Polyarteritis Nodosa

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Cellular components related to Polyarteritis Nodosa according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.81 VCAM1 PRTN3 PECAM1 MSN MPO ICAM1
2 membrane raft GO:0045121 9.62 SELE PRTN3 PECAM1 ICAM1
3 integral component of plasma membrane GO:0005887 9.56 VCAM1 SELP SELL SELE PECAM1 ICAM2
4 extracellular space GO:0005615 9.53 VCAM1 SELP SELE PRTN3 PECAM1 MSN
5 microvillus GO:0005902 9.43 VCAM1 MSN ICAM2
6 uropod GO:0001931 9.37 MSN ICAM2

Biological processes related to Polyarteritis Nodosa according to GeneCards Suite gene sharing:

(show all 19)
# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 10 VCAM1 SELP SELL SELE PECAM1 ICAM2
2 positive regulation of gene expression GO:0010628 9.98 SMAD3 MSN IL6 FGF2 CRP
3 neutrophil degranulation GO:0043312 9.97 SELL PRTN3 PECAM1 MPO ADA2
4 inflammatory response GO:0006954 9.95 SELP SELE MEFV IL6 IFNA2 CRP
5 extracellular matrix organization GO:0030198 9.91 VCAM1 PECAM1 ICAM2 ICAM1 FGF2
6 regulation of immune response GO:0050776 9.89 VCAM1 SMAD3 SELL ICAM2 ICAM1
7 cytokine-mediated signaling pathway GO:0019221 9.85 VCAM1 PRTN3 IL6 IFNA2 ICAM1 FGF2
8 cell-cell adhesion GO:0098609 9.83 VCAM1 PECAM1 ICAM2 ICAM1
9 response to lipopolysaccharide GO:0032496 9.8 VCAM1 SELP SELE MPO ICAM1
10 interferon-gamma-mediated signaling pathway GO:0060333 9.74 VCAM1 ICAM1 HLA-DRB1
11 leukocyte migration GO:0050900 9.63 SELP SELL SELE PECAM1 MSN ICAM1
12 heterophilic cell-cell adhesion via plasma membrane cell adhesion molecules GO:0007157 9.62 VCAM1 SELP SELE ICAM1
13 establishment of endothelial barrier GO:0061028 9.61 PECAM1 MSN ICAM1
14 negative regulation of lipid storage GO:0010888 9.56 IL6 CRP
15 positive regulation of leukocyte tethering or rolling GO:1903238 9.55 SELP SELE
16 neutrophil extravasation GO:0072672 9.54 PRTN3 PECAM1
17 membrane to membrane docking GO:0022614 9.43 VCAM1 MSN ICAM1
18 leukocyte tethering or rolling GO:0050901 9.26 VCAM1 SELP SELL SELE
19 leukocyte cell-cell adhesion GO:0007159 9.1 VCAM1 SELP SELE PECAM1 MSN ICAM1

Molecular functions related to Polyarteritis Nodosa according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integrin binding GO:0005178 9.46 VCAM1 ICAM2 ICAM1 FGF2
2 glycosphingolipid binding GO:0043208 9.26 SELP SELL
3 oligosaccharide binding GO:0070492 9.13 SELP SELL SELE
4 heparin binding GO:0008201 9.1 SELP SELL MPO FGF2 APOH ADA2
Sources

Sources for Polyarteritis Nodosa

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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