PAN
MCID: PLY017
MIFTS: 52

Polyarteritis Nodosa (PAN)

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Nephrological diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Polyarteritis Nodosa

MalaCards integrated aliases for Polyarteritis Nodosa:

Name: Polyarteritis Nodosa 12 76 53 59 37 55 44 15 73
Periarteritis 53 73
Polyarteritis 53 73
Pan 53 59
Küssmaul-Maier Disease 59
Periarteritis Nodosa 59

Characteristics:

Orphanet epidemiological data:

59
polyarteritis nodosa
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:



External Ids:

Disease Ontology 12 DOID:9810
ICD10 33 M30.0 M30.1
ICD9CM 35 446.0
MeSH 44 D010488
NCIt 50 C26847
SNOMED-CT 68 66121003
Orphanet 59 ORPHA767
MESH via Orphanet 45 D010488
UMLS via Orphanet 74 C0031036
ICD10 via Orphanet 34 M30.0
KEGG 37 H01382

Summaries for Polyarteritis Nodosa

NIH Rare Diseases : 53 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 sneddon syndrome. An important gene associated with Polyarteritis Nodosa is ADA2 (Adenosine Deaminase 2), and among its related pathways/superpathways are Innate Immune System and Response to elevated platelet cytosolic Ca2+. The drugs Cyclophosphamide and Azathioprine have been mentioned in the context of this disorder. Affiliated tissues include kidney, lung and skin, and related phenotypes are hematopoietic system and homeostasis/metabolism

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

Wikipedia : 76 Polyarteritis nodosa (PAN), also known as panarteritis nodosa,periarteritis nodosa,Kussmaul disease, or... 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 416)
# Related Disease Score Top Affiliating Genes
1 cutaneous polyarteritis nodosa 34.4 IFNA2 SMAD3
2 sneddon syndrome 32.2 ADA2 APOH
3 churg-strauss syndrome 31.7 MPO PRTN3
4 vasculitis 31.2 APOH CRP IFNA2 MEFV MPO PRTN3
5 kawasaki disease 31.1 CRP IL6 SELE SELL
6 mononeuritis multiplex 30.6 CRP MPO
7 purpura 30.6 APOH CRP IL6
8 colitis 30.6 IL6 MPO SMAD3
9 pneumonia 30.5 CRP IL6 MPO
10 temporal arteritis 30.4 APOH CRP IL6
11 amyloidosis aa 30.4 CRP MEFV
12 peripheral vascular disease 30.4 CRP IL6 SELE
13 psoriatic arthritis 30.3 CRP IL6 SELE
14 retinal artery occlusion 30.3 APOH IL6
15 takayasu arteritis 30.2 CRP IL6 SELE
16 adult-onset still's disease 30.2 CRP IL6 MEFV
17 arteriovenous fistula 30.2 APOH IL6
18 relapsing polychondritis 30.1 CRP IL6
19 intussusception 30.1 CRP IL6
20 pyoderma 30.1 IFNA2 MEFV
21 myocardial infarction 30.1 CRP IL6 MPO SELL
22 granulomatous angiitis 30.1 CRP PRTN3
23 ulcerative colitis 30.1 CRP IL6 MPO PRTN3
24 appendicitis 30.1 CRP IL6 MPO
25 acute pancreatitis 30.1 CRP IL6 MPO
26 ischemic heart disease 30.1 CRP IL6
27 aortitis 30.0 CRP MPO
28 discoid lupus erythematosus 30.0 APOH IFNA2
29 palindromic rheumatism 30.0 CRP MEFV PRTN3
30 henoch-schoenlein purpura 29.9 CRP MEFV MPO
31 pericarditis 29.9 CRP IL6 MEFV
32 cholangitis 29.9 CRP IL6 PRTN3
33 rapidly progressive glomerulonephritis 29.9 CRP MPO PRTN3
34 endocarditis 29.8 APOH CRP IL6
35 intermittent claudication 29.8 APOH CRP IL6
36 vascular disease 29.7 APOH CRP IL6 MPO SELE
37 systemic lupus erythematosus 29.6 APOH CRP IL6 MPO PRTN3 SELE
38 rheumatoid arthritis 29.6 CRP IL6 PRTN3 SELE
39 arteries, anomalies of 29.6 CRP IL6 SELE
40 synovitis 29.6 CRP IL6 SELE
41 hypersensitivity vasculitis 29.3 CRP MEFV MPO PRTN3
42 inflammatory bowel disease 28.7 CRP IL6 MPO PRTN3 SELE SELL
43 polyarteritis nodosa, childhood-onset 12.9
44 systemic polyarteritis nodosa 12.6
45 primary polyarteritis nodosa 12.5
46 secondary polyarteritis nodosa 12.4
47 single-organ polyarteritis nodosa 12.4
48 adenosine deaminase 2 deficiency 11.9
49 retinal vasculitis 11.7
50 autoimmune inner ear disease 11.6

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



Diseases related to Polyarteritis Nodosa

Symptoms & Phenotypes for Polyarteritis Nodosa

MGI Mouse Phenotypes related to Polyarteritis Nodosa:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 IL6 MEFV MPO PRTN3 SELE SELL
2 homeostasis/metabolism MP:0005376 9.56 APOH CRP IL6 MEFV MPO SELE
3 immune system MP:0005387 9.23 CRP IL6 MEFV MPO PRTN3 SELE

Drugs & Therapeutics for Polyarteritis Nodosa

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

(show top 50) (show all 72)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 4,Phase 2,Phase 1,Not Applicable 6055-19-2, 50-18-0 2907
2
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
3
Prednisone Approved, Vet_approved Phase 4,Phase 2,Not Applicable 53-03-2 5865
4
Prednisolone Approved, Vet_approved Phase 4,Not Applicable 50-24-8 5755
5
Methylprednisolone Approved, Vet_approved Phase 4,Not Applicable 83-43-2 6741
6
Prednisolone phosphate Approved, Vet_approved Phase 4,Not Applicable 302-25-0
7
leucovorin Approved Phase 4,Phase 2 58-05-9 6006 143
8
Methylprednisolone hemisuccinate Approved Phase 4,Not Applicable 2921-57-5
9
Methotrexate Approved Phase 4,Phase 2 59-05-2, 1959-05-2 126941
10
Mycophenolic acid Approved Phase 4 24280-93-1 446541
11
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2 59-30-3 6037
12
Prednisolone hemisuccinate Experimental Phase 4,Not Applicable 2920-86-7
13 Immunosuppressive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
14 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
15 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Phase 1,Not Applicable
16 Antimetabolites Phase 4,Phase 3,Phase 2
17 Immunologic Factors Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
18 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2
19 Alkylating Agents Phase 4,Phase 2,Phase 1,Not Applicable
20 Gastrointestinal Agents Phase 4,Not Applicable
21 glucocorticoids Phase 4,Phase 2,Not Applicable
22 Vitamin B9 Phase 4,Phase 2
23 Prednisolone acetate Phase 4,Not Applicable
24 Hormones Phase 4,Phase 2,Not Applicable
25 Anti-Infective Agents Phase 4,Phase 2,Not Applicable
26 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Not Applicable
27 Neuroprotective Agents Phase 4,Not Applicable
28 Antiemetics Phase 4,Not Applicable
29 Vitamin B Complex Phase 4,Phase 2
30 Peripheral Nervous System Agents Phase 4,Phase 2,Not Applicable
31 Folic Acid Antagonists Phase 4,Phase 2
32 Methylprednisolone acetate Phase 4,Not Applicable
33 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Not Applicable
34 Anti-Inflammatory Agents Phase 4,Phase 2,Not Applicable
35 Dermatologic Agents Phase 4,Phase 2,Not Applicable
36 Hormone Antagonists Phase 4,Phase 2,Not Applicable
37 Protective Agents Phase 4,Not Applicable
38 Autonomic Agents Phase 4,Not Applicable
39 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2
40 Folate Phase 4,Phase 2
41 Antitubercular Agents Phase 4
42 Antibiotics, Antitubercular Phase 4
43 Anti-Bacterial Agents Phase 4,Phase 2
44
Lamivudine Approved, Investigational Phase 2 134678-17-4 60825
45
Dapsone Approved, Investigational Phase 2 80-08-0 2955
46
Colchicine Approved Phase 2 64-86-8 6167 2833
47
Naltrexone Approved, Investigational, Vet_approved Phase 2 16590-41-3 5360515
48 Reverse Transcriptase Inhibitors Phase 2
49 Anti-HIV Agents Phase 2
50 Anti-Retroviral Agents Phase 2

Interventional clinical trials:

(show all 26)
# Name Status NCT ID Phase Drugs
1 CHUSPAN PAN BP Treatment of Polyarteritis Nodosa and Microscopic Polyangiitis Without Poor-Prognosis Factors Unknown status NCT00400075 Phase 4 azathioprine;cyclophosphamide
2 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Completed NCT00307671 Phase 4 prednisone, methylprednisolone,cyclophosphamides;Cyclophosphamide, Azathioprine,prednisone,methylprednisolone;Mycophenolate mofetil,methotrexate
3 Association Corticosteroid/Azathioprine in Microscopic Polyangiitis/ Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome) Completed NCT00647166 Phase 3 corticosteroid and azathioprine;corticosteroid and placebo
4 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
5 Lamivudine for Chronic Hepatitis B Completed NCT00001457 Phase 2 Lamivudine
6 Steroids and Methotrexate to Treat Systemic Vasculitis Completed NCT00001256 Phase 2 prednisone and methotrexate
7 A Randomized Multicenter Study for Isolated Skin Vasculitis Recruiting NCT02939573 Phase 2 Colchicine;Dapsone;Azathioprine
8 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Not yet recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
9 Hematopoietic Stem Cell Support in Vasculitis Terminated NCT00278512 Phase 1 Cyclophosphamide;ATG (rabbit);G-CSF;Campath-1h;Fludarabine;Tacrolimus;Mesna
10 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis Unknown status NCT01066208
11 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055 Not Applicable anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone
12 Survey of Patients With Idiopathic Orbital Inflammation Syndrome Unknown status NCT01443000
13 Educational Needs of Patients With Systemic Vasculitis Completed NCT02190929
14 Cyclophosphamide and Prednisone Followed by Methotrexate To Treat Vasculitides Completed NCT00001473
15 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
16 Vasculitis Illness Perception (VIP) Study Completed NCT02190916
17 Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
18 Impact of Vasculitis on Employment and Income Completed NCT02476292
19 Steroid-Induced Osteoporosis in the Pediatric Population - Canadian Incidence Study Completed NCT01663129
20 Determining Disease Activity Biomarkers in Individuals With Polyarteritis Nodosa Recruiting NCT00315406
21 Pediatric Vasculitis Initiative Recruiting NCT02006134
22 One-Time DNA Study for Vasculitis Recruiting NCT01241305
23 VCRC Tissue Repository Recruiting NCT02967068
24 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
25 Vasculitis Pregnancy Registry Recruiting NCT02593565
26 VCRC Patient Contact Registry Patient-Reported Data Validation Study Enrolling by invitation NCT02190942

Search NIH Clinical Center for Polyarteritis Nodosa

Cochrane evidence based reviews: polyarteritis nodosa

Genetic Tests for Polyarteritis Nodosa

Anatomical Context for Polyarteritis Nodosa

MalaCards organs/tissues related to Polyarteritis Nodosa:

41
Kidney, Lung, Skin, Heart, Eye, Liver, Bone

Publications for Polyarteritis Nodosa

Articles related to Polyarteritis Nodosa:

(show top 50) (show all 1171)
# Title Authors Year
1
Severe and Refractory Polyarteritis Nodosa Associated With CECR1 Mutation and Dramatic Response to Infliximab in Adulthood. ( 29916967 )
2018
2
Minocycline-associated polyarteritis nodosa and self-limiting hepatitis: a reminder of a potentially re-emerging culprit in drug-induced autoimmune syndromes. ( 29532736 )
2018
3
Clinical and Laboratory Markers Associated With Relapse in Cutaneous Polyarteritis Nodosa. ( 29955757 )
2018
4
Blue toe syndrome in cutaneous polyarteritis nodosa. ( 29534226 )
2018
5
Adalimumab therapy in hepatitis B virus-negative polyarteritis nodosa: A case report. ( 29923995 )
2018
6
Diagnosis and management of ADA2 deficient polyarteritis nodosa. ( 29624883 )
2018
7
Atypical case of classical polyarteritis nodosa: alveolar hemorrhage and positive antineutrophil cytoplasmic antibodya8c. ( 29162217 )
2018
8
Diagnosis of deficiency of adenosine deaminase 2 with early onset polyarteritis nodosa in an adult patient with a novel compound heterozygous CECR1 mutation. ( 29600946 )
2018
9
Rhabdomyolysis in a Patient with Polyarteritis Nodosa. ( 29021478 )
2018
10
Vasculitis or coronary atherosclerosis? Optical coherence tomography images in polyarteritis nodosa. ( 29652433 )
2018
11
The Role of Renal Angiography in Hepatitis B-related Polyarteritis Nodosa. ( 29515309 )
2018
12
Interobserver reliability of histopathological features for distinguishing between cutaneous polyarteritis nodosa and superficial thrombophlebitis. ( 29675878 )
2018
13
Childhood Polyarteritis Nodosa Presenting With Symmetric Digital Gangrene and Hyperesthesia. ( 29384826 )
2018
14
Catastrophic multiple arterial dissections revealing concomitant polyarteritis nodosa and vascular Elhers-Danlos syndrome. ( 29745881 )
2018
15
Abdominal pain leading to incidental finding of polyarteritis nodosa. ( 29954772 )
2018
16
Acute Necrotizing Vasculitic Neuropathy due to Polyarteritis Nodosa. ( 29896335 )
2018
17
Case Report of Empagliflozin-Induced Cutaneous Polyarteritis Nodosa. ( 29457486 )
2018
18
Cutaneous polyarteritis nodosa resulting from a paclitaxel-eluting balloon angioplasty. ( 29387747 )
2018
19
Thrombosed Aneurysm of the Posterior Inferior Cerebellar Artery and Lateral Medullary Ischemia as the Initial Presentation of Polyarteritis Nodosa: Case Report and Literature Review. ( 29602618 )
2018
20
Polyarteritis Nodosa in Pregnancy. ( 29559879 )
2018
21
Prostate cancer-associated polyarteritis nodosa: improvement of clinical manifestations after prostatectomy. ( 29352851 )
2018
22
Case Report: Polyarteritis nodosa or complicated Henoch-Schonlein purpura (IgAV), a rare case. ( 29770208 )
2018
23
The "Nodes" in Polyarteritis Nodosa-Coronary Artery Aneurysms. ( 29846272 )
2018
24
Spontaneous bilateral perirenal and splenic haematoma in childhood onset polyarteritis nodosa. ( 29950374 )
2018
25
Polyarteritis nodosa in case of familial Mediterranean fever. ( 30511549 )
2018
26
Polyarteritis Nodosa and Membranous Glomerulonephritis: Two Simultaneous Extrahepatic Manifestations of Hepatitis B. ( 29965852 )
2018
27
"Emergency" Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa: How to Face a Rare Entity. ( 30541729 )
2018
28
Orchitis: An unusual presentation of polyarteritis nodosa. ( 30303162 )
2018
29
Recurrent spontaneous renal haemorrhage due to polyarteritis nodosa: a medical cause for a surgical problem. ( 28239941 )
2018
30
Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda. ( 29465365 )
2018
31
Cardiac manifestation of polyarteritis nodosa. ( 29481598 )
2018
32
Ruptured hepatic aneurysm as first presenting symptom of polyarteritis nodosa. ( 29492271 )
2018
33
Hybrid Surgery to Treat Multiple Visceral Aneurysms Secondary to Polyarteritis Nodosa. ( 29984216 )
2018
34
Polyarteritis nodosa limited to the breasts. ( 30051627 )
2018
35
A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts. ( 30099592 )
2018
36
Childhood- versus Adult-Onset Polyarteritis Nodosa Results from the French Vasculitis Study Group Registry. ( 30114520 )
2018
37
Acute Surgical Abdomen Due to Sporadic Polyarteritis Nodosa. ( 30142116 )
2018
38
Nodules developing after radiofrequency ablation of varicosities: A potential clinical and histopathologic mimic of polyarteritis nodosa. ( 30246136 )
2018
39
Immunoglobulin G4-related disease with recurrent uveitis and kidney tumor mimicking childhood polyarteritis nodosa: a rare case report. ( 30414371 )
2018
40
Polyarteritis nodosa: lessons from 25 years of experience. ( 30418125 )
2018
41
Tocilizumab treatment in refractory polyarteritis nodosa: a case report and review of the literature. ( 30465270 )
2018
42
Case Report: Unexpected Benefit of Echocardiography in Childhood Polyarteritis Nodosa. ( 30537767 )
2018
43
Coronary periarteritis associated with multivessel coronary artery disease. ( 29135483 )
2018
44
IgG4-related periaortitis/periarteritis: an under-recognized condition that is potentially life-threatening. ( 30474460 )
2018
45
Reply to Letter to Editor regarding the article, "Polyarteritis nodosa presenting as peripheral vascular disease and acute limb ischemia". ( 28397743 )
2017
46
Association of five-factor score with the mortality in Japanese patients with polyarteritis nodosa. ( 28648103 )
2017
47
Endovascular coil treatment of a coronary artery aneurysm related to polyarteritis nodosa. ( 29145218 )
2017
48
Cytomegalovirus-Induced Polyarteritis Nodosa in a Liver Transplant Recipient. ( 28556523 )
2017
49
Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel. ( 29267446 )
2017
50
Coronary Artery Bypass Grafting in a Patient With Polyarteritis Nodosa. ( 28431718 )
2017

Variations for Polyarteritis Nodosa

Expression for Polyarteritis Nodosa

Search GEO for disease gene expression data for Polyarteritis Nodosa.

Pathways for Polyarteritis Nodosa

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 region GO:0005576 9.7 ADA2 APOH CRP IFNA2 IL6 MPO
2 extracellular space GO:0005615 9.23 ADA2 APOH CRP IFNA2 IL6 MPO
3 azurophil granule lumen GO:0035578 9.13 ADA2 MPO PRTN3

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

# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.56 ADA2 MPO PRTN3 SELL
2 acute-phase response GO:0006953 9.32 CRP IL6
3 leukocyte tethering or rolling GO:0050901 9.16 SELE SELL
4 inflammatory response GO:0006954 9.02 CRP IFNA2 IL6 MEFV SELE
5 negative regulation of lipid storage GO:0010888 8.96 CRP IL6

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

# Name GO ID Score Top Affiliating Genes
1 oligosaccharide binding GO:0070492 8.96 SELE SELL
2 heparin binding GO:0008201 8.92 ADA2 APOH MPO SELL

Sources for Polyarteritis Nodosa

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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