PAN
MCID: PLY017
MIFTS: 62

Polyarteritis Nodosa (PAN)

Categories: Bone diseases, Cardiovascular diseases, Immune diseases, Nephrological diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Polyarteritis Nodosa

MalaCards integrated aliases for Polyarteritis Nodosa:

Name: Polyarteritis Nodosa 11 19 58 75 28 53 5 43 14 16 71 31 33
Periarteritis Nodosa 58 75 33
Polyarteritis 19 75 71
Periarteritis 19 71
Pan 19 58
Pan - [polyarteritis Nodosa] 33
Kussmaul-Maier Disease 58
Panarteritis Nodosa 33
Kussmaul Disease 33
Arteritis Nodosa 33
Polyangiitis 33
Panangiitis 33

Characteristics:


Prevelance:

1-9/100000 (Sweden, France, Norway, Europe) 1-9/1000000 (Australia, Spain) <1/1000000 (Germany) 58

Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare circulatory system diseases
Rare renal diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 11 DOID:9810
ICD9CM 34 446.0
MeSH 43 D010488
NCIt 49 C26847
SNOMED-CT 68 195347004
ICD10 31 M30.0 M30.1
MESH via Orphanet 44 D010488
ICD10 via Orphanet 32 M30.0
UMLS via Orphanet 72 C0031036
Orphanet 58 ORPHA767
ICD11 33 1419332129
UMLS 71 C0031036 C0264988 C0264992

Summaries for Polyarteritis Nodosa

GARD: 19 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.

MalaCards based summary: Polyarteritis Nodosa, also known as periarteritis nodosa, is related to mononeuritis multiplex and familial mediterranean fever. An important gene associated with Polyarteritis Nodosa is ADA2 (Adenosine Deaminase 2), and among its related pathways/superpathways are Integrin Pathway and Cytokine Signaling in Immune system. The drugs Alemtuzumab and Levoleucovorin have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and neutrophil, and related phenotypes are fever and arthralgia

Orphanet: 58 A rare, clinically heterogeneous, systemic disease characterized by necrotizing inflammatory lesions affecting medium-sized blood vessels. It most commonly affects skin, joints, peripheral nerves and the gastrointestinal tract.

Disease Ontology: 11 A vasculitis that is characterized by necrotizing inflammation of medium or small arteries.

Wikipedia: 75 Polyarteritis nodosa (PAN) is a systemic necrotizing inflammation of blood vessels (vasculitis)... more...

Related Diseases for Polyarteritis Nodosa

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 1244)
# Related Disease Score Top Affiliating Genes
1 mononeuritis multiplex 31.6 PRTN3 MPO HLA-DRB1 CRP APOH ADA2
2 familial mediterranean fever 31.4 MEFV IL6 CRP
3 churg-strauss syndrome 31.3 PRTN3 MPO HLA-DRB1
4 microscopic polyangiitis 31.3 PRTN3 MPO HLA-DRB1 ADA2
5 acute kidney failure 31.3 PRTN3 IL6 GPT
6 hepatitis b 31.2 IL6 IFNA2 HLA-DRB1 GPT
7 purpura 31.1 SELP IL6 HLA-DRB1 CRP APOH
8 orchitis 31.1 IL6 IFNA2 CRP
9 viral infectious disease 31.1 IL6 IFNA2 ICAM1 GPT
10 intestinal perforation 31.0 IL6 GPT CRP
11 cryoglobulinemia 31.0 IL6 IFNA2 HLA-DRB1 GPT
12 spondyloarthropathy 1 31.0 PRTN3 IL6 CRP
13 thrombocytosis 31.0 SELP IL6 CRP
14 kawasaki disease 31.0 VCAM1 SELP SELL SELE GPT CRP
15 raynaud disease 30.9 SELE CRP APOH
16 systemic scleroderma 30.9 PRTN3 IL6 HLA-DRB1 CRP
17 granulomatous angiitis 30.9 PRTN3 CRP
18 anterior uveitis 30.9 IL6 HLA-DRB1 CRP
19 collagen disease 30.9 PRTN3 IL6 CRP APOH
20 testicular infarct 30.8 CRP ADA2
21 systemic onset juvenile idiopathic arthritis 30.8 IL6 HLA-DRB1
22 hepatic infarction 30.8 GPT APOH
23 antiphospholipid syndrome 30.8 VCAM1 SELP SELE CRP APOH
24 exanthem 30.8 PRTN3 MPO IL6 GPT CRP
25 cardiac tamponade 30.8 IL6 GPT CRP
26 disseminated intravascular coagulation 30.8 IL6 GPT CRP
27 livedoid vasculitis 30.8 PRTN3 APOH ADA2
28 hepatitis a 30.7 IFNA2 HLA-DRB1 GPT
29 pyelonephritis 30.7 IL6 GPT CRP
30 serum amyloid a amyloidosis 30.7 MEFV IL6 CRP
31 thrombophlebitis 30.7 SELP CRP APOH
32 uveitis 30.7 IL6 IFNA2 ICAM1 HLA-DRB1
33 crescentic glomerulonephritis 30.7 PRTN3 MPO CRP
34 erythema nodosum 30.7 VCAM1 SELE IL6 ICAM1 CRP
35 adult-onset still's disease 30.7 MEFV IL6 CRP
36 retinal artery occlusion 30.7 IL6 ICAM1 CRP APOH
37 limb ischemia 30.7 PECAM1 IL6 CRP
38 pericarditis 30.7 MEFV IL6 CRP
39 pericardial effusion 30.7 IL6 GPT CRP
40 brucellosis 30.7 SELL MPO MEFV IL6 CRP
41 rheumatic fever 30.6 IL6 ICAM1 HLA-DRB1
42 acalculous cholecystitis 30.6 GPT CRP
43 juvenile rheumatoid arthritis 30.6 IL6 HLA-DRB1 CRP
44 pulmonary tuberculosis 30.6 IL6 HLA-DRB1 CRP
45 psoriatic arthritis 30.6 SELE IL6 HLA-DRB1 CRP
46 facial paralysis 30.6 PRTN3 IL6 CRP
47 buerger disease 30.6 VCAM1 ICAM1 CRP APOH
48 kidney disease 30.6 VCAM1 SELE PRTN3 MEFV IL6 ICAM1
49 thrombotic thrombocytopenic purpura 30.6 SELP SELE APOH
50 mononeuropathy 30.6 PRTN3 MPO IL6 CRP

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



Diseases related to Polyarteritis Nodosa

Symptoms & Phenotypes for Polyarteritis Nodosa

Human phenotypes related to Polyarteritis Nodosa:

58 30 (show all 28)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 58 30 Frequent (33%) Frequent (79-30%)
HP:0001945
2 arthralgia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002829
3 myalgia 58 30 Frequent (33%) Frequent (79-30%)
HP:0003326
4 abnormality of the kidney 58 30 Frequent (33%) Frequent (79-30%)
HP:0000077
5 weight loss 58 30 Frequent (33%) Frequent (79-30%)
HP:0001824
6 polyneuritis 58 30 Frequent (33%) Frequent (79-30%)
HP:0031003
7 elevated circulating c-reactive protein concentration 30 Frequent (33%) HP:0011227
8 hypertension 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000822
9 skin ulcer 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0200042
10 subcutaneous nodule 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001482
11 abdominal pain 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002027
12 pericarditis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001701
13 erythema 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0010783
14 abnormality of the gastrointestinal tract 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0011024
15 cutis marmorata 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000965
16 raynaud phenomenon 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0030880
17 sensory axonal neuropathy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003390
18 morphological central nervous system abnormality 30 Occasional (7.5%) HP:0002011
19 abnormality of the eye 58 30 Very rare (1%) Very rare (<4-1%)
HP:0000478
20 cardiomyopathy 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001638
21 pleuritis 58 30 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 elevated c-reactive protein level 58 Frequent (79-30%)
28 abnormality of skin morphology 58 Very frequent (99-80%)

MGI Mouse Phenotypes related to Polyarteritis Nodosa:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.21 APOH CRP ICAM1 IL6 ITGAL ITGB2
2 cellular MP:0005384 10.1 ICAM1 IL6 ITGAL ITGB2 MEFV MSN
3 immune system MP:0005387 10.06 CRP ICAM1 ICAM2 IFNA2 IL6 ITGAL
4 cardiovascular system MP:0005385 10 CRP ICAM1 IL6 ITGB2 MPO MSN
5 respiratory system MP:0005388 9.56 ICAM2 IFNA2 IL6 MSN PECAM1 SELE
6 hematopoietic system MP:0005397 9.5 ICAM1 ICAM2 IFNA2 IL6 ITGAL ITGB2

Drugs & Therapeutics for Polyarteritis Nodosa

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

(show top 50) (show all 120)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Alemtuzumab Approved, Investigational Phase 4 216503-57-0
2
Levoleucovorin Approved, Experimental, Investigational Phase 4 68538-85-2, 58-05-9, 73951-54-9 149436 6006
3
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 4112 126941
4
Povidone K30 Approved, Experimental, Withdrawn Phase 4 9003-39-8 6917 131751496
5
Hydroxychloroquine Approved Phase 4 118-42-3 3652
6
Tofacitinib Approved, Investigational Phase 4 477600-75-2 9926791
7
Rituximab Approved Phase 4 174722-31-7
8
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
9 Dermatologic Agents Phase 4
10 Folic Acid Antagonists Phase 4
11 Folate Phase 4
12 Vitamin B9 Phase 4
13 Vitamin B Complex Phase 4
14 Protein Kinase Inhibitors Phase 4
15 Tin Fluorides Phase 4
16 Antirheumatic Agents Phase 4
17 Antineoplastic Agents, Immunological Phase 4
18 Immunologic Factors Phase 4
19
Alendronic acid Approved Phase 3 121268-17-5, 66376-36-1 2088
20
Etanercept Approved, Investigational Phase 2, Phase 3 185243-69-0
21
Coenzyme M Approved, Investigational Phase 3 3375-50-6 598 23662354
22
Benralizumab Approved, Investigational Phase 3 1044511-01-4
23
Azathioprine Approved Phase 3 446-86-6 2265
24
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
25
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
26
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
27
Prednisolone Approved, Vet_approved Phase 3 50-24-8 4894 5755
28
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
29
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5 1875
30
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 4159 6741
31
Mycophenolic acid Approved, Investigational Phase 3 24280-93-1 446541
32
Cholecalciferol Approved, Nutraceutical, Vet_approved Phase 3 67-97-0, 1406-16-2 5280795 10883523
33
Alfacalcidol Approved, Nutraceutical Phase 3 41294-56-8 5282181
34
Dexchlorpheniramine Experimental, Investigational Phase 3 25523-97-1 33036
35
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7 4897
36
Gusperimus Investigational Phase 3 89149-10-0, 98629-43-7 55362
37 Vitamins Phase 3
38 Trace Elements Phase 3
39 Calciferol Phase 3
40 Micronutrients Phase 3
41 Plasma Substitutes Phase 3
42 Analgesics Phase 2, Phase 3
43 Analgesics, Non-Narcotic Phase 2, Phase 3
44 Hydroxycholecalciferols Phase 3
45 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
46 Pharmaceutical Solutions Phase 3
47 Anti-Asthmatic Agents Phase 3
48 Respiratory System Agents Phase 3
49 Gastrointestinal Agents Phase 2, Phase 3
50 Antimetabolites Phase 3

Interventional clinical trials:

(show top 50) (show all 104)
# 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 Alemtuzumab for ANCA Associated Refractory Vasculitis - a Study of Safety and Efficacy Unknown status NCT01405807 Phase 4 Alemtuzumab
3 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
4 Short-Course Glucocorticoids and Rituximab in ANCA-Associated Vasculitis Completed NCT02169219 Phase 4 Glucocorticoids;Rituximab
5 A Study Evaluating the Safety and Efficacy of Rituximab in Combination With Glucocorticoids in Participants With Wegener's Granulomatosis or Microscopic Polyangitis Completed NCT02115997 Phase 4 Methylprednisolone;Prednisone;Rituximab
6 Hydroxychloroquine in ANCA Vasculitis Evaluation - A Multicentre, Randomised, Double-blind, Placebo-controlled Trial Recruiting NCT04316494 Phase 4 Hydroxychloroquine;Placebo
7 MAINtenance of Remission With RITuximab Versus Azathioprine for Patients With Newly-diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. A Prospective, Randomized, Controlled, Double-blind Study: the MAINRITSEG Trial Recruiting NCT03164473 Phase 4 Rituximab;Azathioprine;Placebo-rituximab;Placebo-azathioprine
8 Randomized Trial of Tofacitinib Versus Methotrexate for Maintenance Therapy in Granulomatosis With Polyangiitis Recruiting NCT04944524 Phase 4 Tofacitinib;Methotrexate
9 Low-dose Glucocorticoids Plus Rituximab Versus High-dose Glucocorticoids Plus Rituximab for Remission Induction in ANCA-associated Vasculitis; a Multicentre, Open Label, Randomised Control Trial Active, not recruiting NCT02198248 Phase 4 Rituximab;Glucocorticoids
10 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Terminated NCT02626845 Phase 4 Rituximab
11 Assessment of the Safety, Side Effects and Efficacy of Interventional Cryotherapy for the Eradication of Benign Airway Disease("ICE THE BAD") Terminated NCT00747461 Phase 4
12 Comparative Study of the Efficacy of Induction Therapy With Cyclophosphamide or Mycophenolate Mofetil for Non-Life-Threatening Relapses of PR3- or MPO-ANCA Associated Vasculitis Unknown status NCT00103792 Phase 3 mycophenolate mofetil;cyclophosphamide
13 Treatment of ANCA-Associated Vasculitides : Corticosteroids and Pulse Cyclophosphamide Followed by Maintenance Therapy With Methotrexate or Azathioprine: a Prospective Multicenter Randomized Trial Unknown status NCT00349674 Phase 3 Azathioprine: 2 mg/kg/day;methotrexate 0.3 mg/kg/week, to a maximum and optimal dose of 25 mg/week
14 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
15 Plasma Exchange and Glucocorticoid Dosing in the Treatment of Anti-neutrophil Cytoplasm Antibody Associated Vasculitis: an International Randomized Controlled Trial Completed NCT00987389 Phase 3 Glucocorticoids [Standard Dose];Glucocorticoids [Reduced Dose]
16 MAINtenance of Remission Using RITuximab in Systemic ANCA-associated Vasculitis II Completed NCT01731561 Phase 3 Rituximab (Arm B);Rituximab (Arm A)
17 MAINtenance of Remission Using RITuximab in Systemic ANCA-associated Vasculitis Completed NCT00748644 Phase 3 Rituximab;Azathioprine
18 Randomized Trial of Intravenous Pulse Versus Oral Continuous Cyclophosphamide for Induction of Remission in Systemic ANCA-Associated Vasculitides Completed NCT00430105 Phase 2, Phase 3 cyclophosphamide
19 An International, Open Label, Randomised Controlled Trial Comparing Rituximab With Azathioprine as Maintenance Therapy in Relapsing ANCA-associated Vasculitis Completed NCT01697267 Phase 3 Azathioprine
20 Rituximab Therapy for the Induction of Remission and Tolerance in ANCA-Associated Vasculitis (ITN021AI) Completed NCT00104299 Phase 2, Phase 3 Rituximab plus cyclophosphamide placebo (rituximab group);Cyclophosphamide plus rituximab placebo (control group);Azathioprine;Methylprednisolone (or other glucocorticoid);Prednisone
21 A Double-blind, Randomised, Placebo-controlled Study to Investigate the Efficacy and Safety of Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis in Subjects Receiving Standard of Care Therapy Completed NCT02020889 Phase 3 Placebo
22 Evaluation of Rituximab-based Regimen Compared to Conventional Therapeutic Strategy For Remission Induction In Patients With Newly-Diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. Prospective, Randomized, Controlled, Double-blind Study Completed NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
23 A Phase 3, Multi-Center, Multinational, Randomized, Double-Blind, Study to Evaluate the Efficacy and Safety of Belimumab (HGS1006) in Combination With Azathioprine for the Maintenance of Remission in Wegener's Granulomatosis and Microscopic Polyangiitis Completed NCT01663623 Phase 3 Azathioprine
24 Prevention of Glucocorticoid-Induced Osteoporosis in Patients With Rheumatic Diseases. The STOP-Study: a Randomized Placebo Controlled Trial With Alendronate Versus Alfacalcidol. Completed NCT00138983 Phase 3 Alendronate versus alfacalcidol (1-alpha OH vitamin D)
25 Wegener's Granulomatosis Etanercept Trial (WGET) Completed NCT00005007 Phase 2, Phase 3 Etanercept
26 Abatacept (CTLA4-Ig) for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) (ABROGATE) Recruiting NCT02108860 Phase 3 Abatacept;placebo
27 A 52-week, Randomized, Double-blind, Double-dummy, Parallel-group, Multi-centre, Non-inferiority Study to Investigate the Efficacy and Safety of Depemokimab Compared With Mepolizumab in Adults With Relapsing or Refractory Eosinophilic Granulomatosis With Polyangiitis (EGPA) Receiving Standard of Care (SoC) Therapy Recruiting NCT05263934 Phase 3 Placebo matching mepolizumab;Placebo matching depemokimab
28 Evaluation of MEpolizumab-based Regimen Compared to Conventional Therapeutic Strategy For Remission Induction In Patients With Eosinophilic Granulomatosis With Polyangiitis. Prospective, Randomized, Controlled, Double-blind Study Recruiting NCT05030155 Phase 3 Mepolizumab;cyclophosphamide/azathioprine;Placebo
29 The Assessment of Prednisone In Remission Trial (TAPIR) - Centers of Excellence Approach Recruiting NCT01940094 Phase 3 5 mg Prednisone;0 mg Prednisone
30 Efficacy and Safety of Rituximab in the Treatment of Good Prognosis Microscopic Polyangiitis Active, not recruiting NCT03920722 Phase 3 Rituximab;placebo
31 A Randomized, Double-blind, Active-controlled 52-week Study With an Open-label Extension to Evaluate the Efficacy and Safety of Benralizumab Compared to Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA) in Patients Receiving Standard of Care Therapy Active, not recruiting NCT04157348 Phase 3
32 Mepolizumab Long-term Access Programme for Subjects Who Participated in Study MEA115921 (Placebo-controlled Study of Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis in Subjects Receiving Standard-of-care Therapy) Active, not recruiting NCT03298061 Phase 3 Mepolizumab;Prednisolone
33 The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach Active, not recruiting NCT01933724 Phase 3 5 mg prednisone;0 mg prednisone
34 Randomised, Evaluator-Blinded, Multicentre, International, Parallel-Group, Active-Controlled Clinical Trial of Gusperimus Versus Conventional Therapy in Relapse of Granulomatosis With Polyangiitis (Wegener's Granulomatosis) SPARROW Study - SPAnidin in Relapsing GRanulomatosis With POlyangiitis Wegener's Granulomatosis) Terminated NCT01446211 Phase 3 Gusperimus + glucocorticoids;cyclophosphamide followed by methotrexate (azathioprine) + glucocorticoids or methotrexate (azathioprine) + glucocorticoids
35 Randomised Trial of Plasma Exchange or High Dose Methyl Prednisolone as Adjunctive Therapy for Severe Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3 Intravenous methyl prednisolone;Methyl prednisolone
36 Intravenous Immunoglobulin After Relapse in Vasculitis (Microscopic Polyangiitis, Wegener's Granulomatosis and SHURG-STRAUSS Syndrome) During and After Corticosteroids and Immunosuppressant Therapies a Multicenter Prospective Trial Terminated NCT00307658 Phase 3 Intravenous immunoglobulins (human immunoglobulins G)
37 Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis Terminated NCT00307645 Phase 3 Cyclophosphamide;Mycophenolate mofetil;Azathioprine;Prednisone (and methylprednisolone)
38 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
39 Open-Label, to Evaluate the Efficacy and Safety of Reslizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA) Study: RITE Study Unknown status NCT02947945 Phase 2 Reslizumab
40 Lamivudine for Chronic Hepatitis B Completed NCT00001457 Phase 2 Lamivudine
41 A Randomized, Double-blind, Double-dummy, Active-controlled, Multicenter, 2-part Phase II Study on Replacement of Steroids by IFX-1 in Active Granulomatosis With Polyangiitis (GPA) and Microscopic Polyangiitis (MPA) Completed NCT03895801 Phase 2 IFX-1;Placebo-IFX-1;Glucocorticoid (GC);Placebo-Glucocorticoid (Placebo-GC)
42 A Phase IIA, International, Multicenter, Open-label, Uncontrolled Study to Evaluate The Safety And Pharmacokinetics of 4 × 375 mg/m2 Intravenous Rituximab in Pediatric Patients With Severe Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis Completed NCT01750697 Phase 2 Rituximab
43 Phase II Pilot Cohort Study to Investigate the Safety and Efficacy of Infliximab as Additional Therapy in the Treatment if Anti-Neutrophil Cytoplasm Antibody Associated Vasculitis Completed NCT00753103 Phase 2 Cyclophosphamide;Prednisolone;Azathioprine;Mycophenolate mofetil;Methylprednisolone
44 Induction of Regulatory t Cells by Low Dose IL2 in Autoimmune and Inflammatory Diseases: a Transnosographic Approach Completed NCT01988506 Phase 2 Interleukin 2
45 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2 Gusperimus
46 A Multi-Center, Open-label Pilot Study of Abatacept (CTLA4-Ig) in the Treatment of Mild Relapsing Wegener's Granulomatosis Completed NCT00468208 Phase 1, Phase 2 Abatacept
47 An Open Trial of the Efficacy of Glucocorticoids and Methotrexate (MTX) in the Treatment of Systemic Vasculitis Completed NCT00001256 Phase 2 prednisone and methotrexate
48 A Randomized Trial Examining the Use of Daclizumab in Wegener's Granulomatosis Completed NCT00040248 Phase 2 Daclizumab
49 Treatment of Wegener's Granulomatosis With Cyclophosphamide Completed NCT00001155 Phase 2 cyclophosphamide
50 A Randomized Trial Comparing Methotrexate Versus Mycophenolate Mofetil for Remission Maintenance in Wegener's Granulomatosis and Related Vasculitides Completed NCT00004567 Phase 2 Mycophenolate Mofetil

Search NIH Clinical Center for Polyarteritis Nodosa

Cochrane evidence based reviews: polyarteritis nodosa

Genetic Tests for Polyarteritis Nodosa

Genetic tests related to Polyarteritis Nodosa:

# Genetic test Affiliating Genes
1 Polyarteritis Nodosa 28

Anatomical Context for Polyarteritis Nodosa

Organs/tissues related to Polyarteritis Nodosa:

MalaCards : Lung, Skin, Neutrophil, Heart, Bone, Kidney, T Cells

Publications for Polyarteritis Nodosa

Articles related to Polyarteritis Nodosa:

(show top 50) (show all 9820)
# 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. 53 62
19796529 2009
2
Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa. 53 62
18035151 2007
3
Cytomegalovirus infection in systemic necrotizing vasculitis: causative agent or opportunistic infection? 53 62
11149660 2000
4
Clinical, laboratory and molecular characteristics of children with Familial Mediterranean Fever-associated vasculitis. 53 62
10709887 2000
5
[A reversible posterior leukoencephalopathy syndrome in a patient with classical polyarteritis nodosa]. 53 62
9146079 1997
6
Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa. 53 62
7754755 1995
7
[Autoantibodies associated with vasculitis]. 53 62
7933581 1994
8
Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges. 53 62
7912504 1994
9
Elevations of neutrophil proteinase 3 in serum of patients with Wegener's granulomatosis and polyarteritis nodosa. 53 62
7907475 1994
10
Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. 53 62
1466365 1992
11
[Antineutrophil cytoplasmic antibodies--ANCA]. 53 62
1464072 1992
12
Myocardial Involvement in Systemic Autoimmune Rheumatic Diseases. 62
36424026 2023
13
A "Leopard Man" Aspect on 18 F-FDG PET/CT Revealing a VEXAS Syndrome. 62
36252945 2023
14
Structured histopathology and laboratory evidence in nasal polyposis with different pathogenesis. 62
36257231 2023
15
Immunoadsorption and plasmapheresis at the ICU - A description of the frequencies and indications in a single center experience and a case report. 62
36468348 2022
16
Antineutrophil Cytoplasm Antibody-Associated Vasculitides Valvular Impairment: Multicenter Retrospective Study and Systematic Review of the Literature. 62
35840158 2022
17
Granulomatosis with polyangiitis: Common and uncommon presentations. 62
36125112 2022
18
New-onset eosinophilic granulomatosis with polyangiitis in 2 patients during treatment with IL-5 pathway inhibitors. 62
36125736 2022
19
Localised presentation of granulomatosis with polyangiitis: The great masquerade. 62
35907569 2022
20
Epidemiology of granulomatosis with polyangiitis and microscopic polyangiitis in adults in France. 62
36108505 2022
21
Adult case of chronic granulomatous disease mimicking granulomatosis with polyangiitis. 62
36455104 2022
22
Eosinophilic granulomatosis with polyangiitis. 62
36259957 2022
23
Eosinophilic Granulomatosis With Polyangiitis With a Pathologically Proven Calcified Lung Nodule. 62
36455937 2022
24
[18F]FDG PET/CT guided biopsy confirms diagnosis of granulomatosis with polyangiitis. 62
35977670 2022
25
Sequential rituximab and mepolizumab in eosinophilic granulomatosis with polyangiitis (EGPA): a European multicentre observational study. 62
35850947 2022
26
Missing Orbital Bones in a Patient with Granulomatosis with Polyangiitis. 62
35773074 2022
27
Extravascular Necrotizing Granuloma: A Diagnostic Clue for Eosinophilic Granulomatosis With Polyangiitis. 62
35705238 2022
28
Cutaneous manifestations of antineutrophil cytoplasmic antibody-associated vasculitis (AAV): a concise review with emphasis on clinical and histopathologic correlation. 62
35599359 2022
29
Uncommon onset manifestations without renal involvement in microscopic polyangiitis: A case report. 62
36420356 2022
30
Clinical patterns and risk factors in scleritis: a multicentric study in Colombia. 62
35796822 2022
31
[Renal manifestations in vasculitides of small and medium-sized vessels]. 62
36331616 2022
32
Updates of ocular involvement in granulomatosis with polyangiitis. 62
36473987 2022
33
Retrospective assessment of porcine circovirus 3 (PCV-3) in formalin-fixed, paraffin-embedded tissues from pigs affected by different clinical-pathological conditions. 62
36471405 2022
34
Images in Vascular Medicine: Rapid limb deterioration due to polyarteritis nodosa. 62
36190788 2022
35
Clinical image: ultrasound findings and magnetic resonance imaging comparison in the muscular involvement in polyarteritis nodosa. 62
36471118 2022
36
Cutaneous polyarteritis nodosa diagnosis and treatment: A retrospective case series. 62
35714785 2022
37
Letter from the Editor: Management of cutaneous polyarteritis nodosa. 62
35643240 2022
38
Predictive role of red blood cell distribution width to platelet ratio combined mean platelet volume in patients with microscopic polyangiitis. 62
36472404 2022
39
Correction to: Immunoglobulin G4-related coronary periarteritis: a systematic literature review with a case series. 62
35951274 2022
40
A Novel Antineutrophil Extracellular Trap Antibody Targeting Myosin Light Chain 6 in Microscopic Polyangiitis. 62
35705243 2022
41
Hepatic artery pseudoaneurysms and hepatic ischaemic injury: a rare complication of polyarteritis nodosa. 62
36450409 2022
42
Reporting complete heart block in a patient with polyarteritis nodosa after COVID-19 vaccination. 62
36347818 2022
43
An adult case of suspected A20 haploinsufficiency mimicking polyarteritis nodosa. 62
35595232 2022
44
Coronary Artery Aneurysm Rupture in a Patient With Polyarteritis Nodosa. 62
36444190 2022
45
The spectrum of cutaneous polyarteritis nodosa. A case report of two contrasting cases and review of the literature. 62
36447655 2022
46
Psychometric properties of outcome measurement instruments for ANCA-associated vasculitis: a systematic literature review. 62
35293985 2022
47
A Report of 2 Cases of Kidney Involvement in ADA2 Deficiency: Different Disease Phenotypes and the Tissue Response to Type I Interferon. 62
35817275 2022
48
Intravenous immunoglobulin for chronic residual peripheral neuropathy in microscopic polyangiitis: A multicentre randomised double-blind trial. 62
36346309 2022
49
Association between Serum Biomarkers and Peripheral Neuropathy in Microscopic Polyangiitis. 62
36362162 2022
50
Coexistence of relapsing polychondritis and eosinophilic granulomatous with polyangiitis: A rare entity. 62
36373442 2022

Variations for Polyarteritis Nodosa

ClinVar genetic disease variations for Polyarteritis Nodosa:

5
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 ADA2 NM_001282225.2(ADA2):c.-46-108_542+142del DEL Likely Pathogenic
973523 GRCh37: 22:17687819-17690721
GRCh38: 22:17206929-17209831

Expression for Polyarteritis Nodosa

Search GEO for disease gene expression data for Polyarteritis Nodosa.

Pathways for Polyarteritis Nodosa

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

(show all 29)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.33 VCAM1 PECAM1 MSN ITGB2 ITGAL ICAM2
2
Show member pathways
13.25 VCAM1 PRTN3 MSN ITGB2 IL6 IFNA2
3
Show member pathways
13.19 SELP SELL SELE PRTN3 PECAM1 ITGB2
4
Show member pathways
13.08 VCAM1 SELL PRTN3 PECAM1 MSN MPO
5
Show member pathways
12.67 VCAM1 PECAM1 ITGB2 ITGAL ICAM2 ICAM1
6 12.52 VCAM1 PECAM1 MPO ITGB2 ICAM2
7
Show member pathways
12.5 SELP SELL SELE ITGB2 ICAM2 ICAM1
8
Show member pathways
12.41 VCAM1 MSN ITGB2 ITGAL ICAM1
9
Show member pathways
12.08 SELP SELL SELE PECAM1 ITGB2 ITGAL
10
Show member pathways
12.02 ICAM1 ICAM2 ITGAL ITGB2 PECAM1 VCAM1
11 11.98 ICAM1 ICAM2 ITGB2 VCAM1
12 11.92 SELL MPO ITGAL IL6 IFNA2
13 11.91 VCAM1 ITGB2 IL6 ICAM1
14 11.86 VCAM1 SELL ITGB2 ITGAL ICAM2 ICAM1
15 11.84 VCAM1 ITGB2 ITGAL IL6
16 11.71 PECAM1 IL6 IFNA2 ICAM1
17
Show member pathways
11.7 IL6 HLA-DRB1 CRP
18 11.7 SELE IL6 ICAM1
19 11.68 ITGB2 ITGAL IL6
20 11.67 SELL IL6 IFNA2
21 11.66 CRP IL6 ITGB2 SELL
22
Show member pathways
11.6 VCAM1 SELE IL6 ICAM1
23 11.59 VCAM1 IL6 ICAM1
24 11.5 VCAM1 SELE ICAM1
25 11.31 VCAM1 SELE ICAM1
26 11.2 SELP ITGB2 IL6 ICAM1
27 11.2 VCAM1 ITGB2 ITGAL ICAM2 ICAM1
28 10.9 VCAM1 MPO ICAM1
29 10.82 VCAM1 ITGB2 ITGAL ICAM2 ICAM1

GO Terms for Polyarteritis Nodosa

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

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 10.45 VCAM1 PRTN3 PECAM1 MSN MPO ITGB2
2 plasma membrane GO:0005886 10.23 HLA-DRB1 ICAM1 ICAM2 PECAM1 SELE SELL
3 plasma membrane GO:0005887 10.23 HLA-DRB1 ICAM1 ICAM2 PECAM1 SELE SELL
4 cell surface GO:0009986 10.17 APOH HLA-DRB1 ICAM1 ITGAL ITGB2 MSN
5 membrane raft GO:0045121 9.97 SELE PRTN3 PECAM1 ITGB2 ICAM1
6 external side of plasma membrane GO:0009897 9.8 VCAM1 SELP SELE ITGB2 ITGAL ICAM1
7 extracellular space GO:0005615 9.78 ADA2 APOH CRP GPT HLA-DRB1 ICAM1
8 integrin alphaL-beta2 complex GO:0034687 9.56 ITGB2 ITGAL

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

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 10.27 CRP IFNA2 IL6 ITGAL ITGB2 MEFV
2 cell adhesion GO:0007155 10.22 VCAM1 SELP SELL SELE PECAM1 ITGB2
3 response to lipopolysaccharide GO:0032496 10.14 VCAM1 SELP SELE MPO
4 cell-cell adhesion GO:0098609 10.13 VCAM1 PECAM1 ITGB2 ITGAL ICAM2 ICAM1
5 phagocytosis GO:0006909 10 ITGAL ITGB2 PECAM1 PRTN3
6 cell adhesion mediated by integrin GO:0033627 9.99 ITGB2 ITGAL ICAM1
7 establishment of endothelial barrier GO:0061028 9.95 PECAM1 MSN ICAM1
8 membrane to membrane docking GO:0022614 9.88 VCAM1 MSN ICAM1
9 leukocyte tethering or rolling GO:0050901 9.86 VCAM1 SELP SELL SELE
10 heterophilic cell-cell adhesion via plasma membrane cell adhesion molecules GO:0007157 9.85 ICAM1 ITGAL SELE SELP VCAM1
11 neutrophil extravasation GO:0072672 9.83 PRTN3 PECAM1
12 T cell activation via T cell receptor contact with antigen bound to MHC molecule on antigen presenting cell GO:0002291 9.8 ITGAL ICAM1
13 leukocyte cell-cell adhesion GO:0007159 9.58 VCAM1 SELP SELL SELE PECAM1 MSN
14 leukocyte migration GO:0050900 9.5 SELP SELL SELE MSN ICAM1

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

# Name GO ID Score Top Affiliating Genes
1 heparin binding GO:0008201 9.96 SELP SELL MPO APOH ADA2
2 cell adhesion molecule binding GO:0050839 9.76 ITGAL ITGB2 MSN VCAM1
3 glycosphingolipid binding GO:0043208 9.67 SELP SELL
4 integrin binding GO:0005178 9.65 VCAM1 ITGB2 ITGAL ICAM2 ICAM1
5 ICAM-3 receptor activity GO:0030369 9.62 ITGB2 ITGAL
6 oligosaccharide binding GO:0070492 9.1 SELP SELL SELE

Sources for Polyarteritis Nodosa

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
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