MCID: PLY017
MIFTS: 51

Polyarteritis Nodosa

Categories: Rare diseases, Neuronal diseases, Cardiovascular diseases, Nephrological diseases, Bone 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
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 Toll-like Receptor Signaling Pathway and Folate Metabolism. The drugs Methotrexate and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include kidney, skin and lung, and related phenotypes are Decreased viability ratio and immune system

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 339)
# Related Disease Score Top Affiliating Genes
1 cutaneous polyarteritis nodosa 34.9 IFNA2 SMAD3
2 sneddon syndrome 32.0 ADA2 APOH
3 churg-strauss syndrome 31.6 MPO PRTN3
4 amyloidosis aa 30.9 CRP MEFV
5 mononeuritis multiplex 30.9 CRP MPO
6 kawasaki disease 30.9 CRP IL6 SELE
7 pyoderma gangrenosum 30.6 IFNA2 MEFV
8 pyoderma 30.5 IFNA2 MEFV
9 brucellosis 30.5 CRP IL6 MEFV
10 relapsing polychondritis 30.1 CRP IL6
11 palindromic rheumatism 30.0 CRP MEFV PRTN3
12 adult-onset still's disease 29.9 CRP IL6 MEFV
13 discoid lupus erythematosus 29.8 APOH IFNA2
14 peripheral vascular disease 29.8 CRP IL6 SELE
15 colitis 29.8 IL6 MPO SMAD3
16 ulcerative colitis 29.7 CRP IL6 MPO
17 pneumonia 29.7 CRP IL6 MPO
18 psoriatic arthritis 29.7 CRP IL6 SELE
19 takayasu arteritis 29.7 CRP IL6 SELE
20 rapidly progressive glomerulonephritis 29.6 CRP MPO PRTN3
21 arteriovenous fistula 29.6 APOH IL6
22 appendicitis 29.5 CRP IL6 MPO
23 cholangitis 29.4 CRP IL6 PRTN3
24 temporal arteritis 29.3 APOH CRP IL6
25 acute pancreatitis 29.3 CRP IL6 MPO
26 intermittent claudication 28.8 APOH CRP IL6
27 endocarditis 28.8 APOH CRP IL6
28 vasculitis 28.8 APOH CRP IFNA2 MEFV MPO PRTN3
29 rheumatoid arthritis 28.4 CRP IL6 PRTN3 SELE
30 wegener granulomatosis 28.1 CRP IL6 MPO PRTN3 SELE
31 vascular disease 27.5 APOH CRP IL6 MPO SELE
32 inflammatory bowel disease 26.9 CRP IL6 MPO PRTN3 SELE SMAD3
33 systemic lupus erythematosus 26.5 APOH CRP IL6 MPO PRTN3 SELE
34 polyarteritis nodosa, childhood-onset 12.7
35 systemic polyarteritis nodosa 12.5
36 primary polyarteritis nodosa 12.4
37 secondary polyarteritis nodosa 12.3
38 single-organ polyarteritis nodosa 12.3
39 adenosine deaminase 2 deficiency 11.8
40 retinal vasculitis 11.5
41 autoimmune inner ear disease 11.5
42 relapsing fever 10.7 CRP MEFV
43 hepatitis 10.7
44 penile disease 10.6 IFNA2 SMAD3
45 pharyngitis 10.6 CRP MEFV
46 muckle-wells syndrome 10.6 CRP MEFV
47 eosinophilic granulomatosis with polyangiitis 10.6
48 peyronie's disease 10.6 IFNA2 SMAD3
49 hepatitis b 10.6
50 dysentery 10.6 CRP IFNA2

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



Diseases related to Polyarteritis Nodosa

Symptoms & Phenotypes for Polyarteritis Nodosa

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 9.1 APOH IFNA2 MEFV MPO SELE SMAD3

MGI Mouse Phenotypes related to Polyarteritis Nodosa:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.17 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 60)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 4,Phase 2 1959-05-2, 59-05-2 126941
2
Cyclophosphamide Approved, Investigational Phase 4,Phase 2,Not Applicable 50-18-0, 6055-19-2 2907
3
Prednisolone Approved, Vet_approved Phase 4,Not Applicable 50-24-8 5755
4
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
5
Methylprednisolone Approved, Vet_approved Phase 4,Not Applicable 83-43-2 6741
6
Prednisone Approved, Vet_approved Phase 4,Phase 2,Not Applicable 53-03-2 5865
7
Mycophenolic acid Approved Phase 4 24280-93-1 446541
8
Mycophenolate mofetil Approved, Investigational Phase 4 128794-94-5 5281078
9
leucovorin Approved, Nutraceutical Phase 4,Phase 2 58-05-9 143 6006
10
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2 59-30-3 6037
11 Prednisolone acetate Phase 4,Not Applicable
12 Alkylating Agents Phase 4,Phase 2,Not Applicable
13 glucocorticoids Phase 4,Phase 2,Not Applicable
14 Methylprednisolone acetate Phase 4,Not Applicable
15 Methylprednisolone Hemisuccinate Phase 4,Not Applicable
16 Folic Acid Antagonists Phase 4,Phase 2
17 Gastrointestinal Agents Phase 4,Not Applicable
18 Neuroprotective Agents Phase 4,Not Applicable
19 Hormone Antagonists Phase 4,Phase 2,Not Applicable
20 Dermatologic Agents Phase 4,Phase 2,Not Applicable
21 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2
22 Hormones Phase 4,Phase 2,Not Applicable
23 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Not Applicable
24 Peripheral Nervous System Agents Phase 4,Not Applicable
25 Vitamin B Complex Phase 4,Phase 2
26 Immunosuppressive Agents Phase 4,Phase 3,Phase 2,Not Applicable
27 Prednisolone hemisuccinate Phase 4,Not Applicable
28 Prednisolone phosphate Phase 4,Not Applicable
29 Anti-Infective Agents Phase 4,Phase 2,Not Applicable
30 Antiemetics Phase 4,Not Applicable
31 Protective Agents Phase 4,Not Applicable
32 Anti-Inflammatory Agents Phase 4,Phase 2,Not Applicable
33 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Not Applicable
34 Antimetabolites Phase 4,Phase 3,Phase 2
35 Autonomic Agents Phase 4,Not Applicable
36 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2
37 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Not Applicable
38 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Not Applicable
39 Anti-Bacterial Agents Phase 4,Phase 2
40 Antibiotics, Antitubercular Phase 4
41 Antitubercular Agents Phase 4
42 Folate Nutraceutical Phase 4,Phase 2
43 Vitamin B9 Nutraceutical Phase 4,Phase 2
44
Lamivudine Approved, Investigational Phase 2 134678-17-4 60825
45
Colchicine Approved Phase 2 64-86-8 6167 2833
46
Dapsone Approved, Investigational Phase 2 80-08-0 2955
47 Reverse Transcriptase Inhibitors Phase 2
48 Anti-HIV Agents Phase 2
49 Anti-Retroviral Agents Phase 2
50 Antiviral 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
10 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
11 Educational Needs of Patients With Systemic Vasculitis Completed NCT02190929
12 Cyclophosphamide and Prednisone Followed by Methotrexate To Treat Vasculitides Completed NCT00001473
13 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
14 Vasculitis Illness Perception (VIP) Study Completed NCT02190916
15 Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
16 Impact of Vasculitis on Employment and Income Completed NCT02476292
17 Steroid-Induced Osteoporosis in the Pediatric Population - Canadian Incidence Study Completed NCT01663129
18 Determining Disease Activity Biomarkers in Individuals With Polyarteritis Nodosa Recruiting NCT00315406
19 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis Recruiting NCT01066208
20 Pediatric Vasculitis Initiative Recruiting NCT02006134
21 One-Time DNA Study for Vasculitis Recruiting NCT01241305
22 VCRC Tissue Repository Recruiting NCT02967068
23 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
24 Vasculitis Pregnancy Registry Recruiting NCT02593565
25 Survey of Patients With Idiopathic Orbital Inflammation Syndrome Active, not recruiting NCT01443000
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, Skin, Lung, Eye, Heart, Liver, Bone

Publications for Polyarteritis Nodosa

Articles related to Polyarteritis Nodosa:

(show top 50) (show all 787)
# 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 and Membranous Glomerulonephritis: Two Simultaneous Extrahepatic Manifestations of Hepatitis B. ( 29965852 )
2018
26
Reply to Letter to Editor regarding the article, "Polyarteritis nodosa presenting as peripheral vascular disease and acute limb ischemia". ( 28397743 )
2017
27
Association of five-factor score with the mortality in Japanese patients with polyarteritis nodosa. ( 28648103 )
2017
28
Endovascular coil treatment of a coronary artery aneurysm related to polyarteritis nodosa. ( 29145218 )
2017
29
Cytomegalovirus-Induced Polyarteritis Nodosa in a Liver Transplant Recipient. ( 28556523 )
2017
30
Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel. ( 29267446 )
2017
31
Coronary Artery Bypass Grafting in a Patient With Polyarteritis Nodosa. ( 28431718 )
2017
32
A Case of Polyarteritis Nodosa Presenting as Rapidly Progressing Intermittent Claudication of Right Leg. ( 29109739 )
2017
33
Rare and unusual case of polyarteritis nodosa involving the gastrointestinal tract leading to bowel gangrene. ( 28062430 )
2017
34
ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: a multicentre national study. ( 28522451 )
2017
35
Acute kidney failure due to polyarteritis nodosa. ( 27939915 )
2017
36
A case of cutaneous polyarteritis nodosa with PR3-ANCA. ( 28681726 )
2017
37
The changing face of polyarteritis nodosa and necrotizing vasculitis. ( 28490787 )
2017
38
Deficiency of Adenosine Deaminase 2 (DADA2), an Inherited Cause of Polyarteritis Nodosa and a Mimic of Other Systemic Rheumatologic Disorders. ( 28983775 )
2017
39
The histopathology of cutaneous polyarteritis nodosa and its relationship with lymphocytic thrombophilic arteritis. ( 28198027 )
2017
40
Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis with Polyangiitis, Microscopic Polyangiitis or Polyarteritis Nodosa without Poor Prognosis Factors A Randomized-Controlled Trial. ( 28678392 )
2017
41
Multiple small intestinal perforations in a patient with Hepatitis B Virus-associated Polyarteritis Nodosa. ( 28878877 )
2017
42
Cutaneous polyarteritis nodosa localized to a region of lymphedema secondary to Streptococcus viridans cellulitis and multiple surgeries. ( 27862152 )
2017
43
Treatment of polyarteritis nodosa with tocilizumab: a new therapeutic approach? ( 28879047 )
2017
44
Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling. ( 28943380 )
2017
45
Involvement of the Peripheral Nervous System in Polyarteritis Nodosa and Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis. ( 29061248 )
2017
46
Asymptomatic cutaneous polyarteritis nodosa: treatment options and therapeutic guidelines. ( 28961286 )
2017
47
Correction:<i>Gangrenous digital infarcts in a severe case of cutaneous polyarteritis nodosa</i>. ( 29275379 )
2017
48
Refractory Psoriatic Arthritis and Polyarteritis Nodosa: Co-occurrence in a Patient with Undiagnosed HIV. ( 29738130 )
2017
49
Reclassification of polyarteritis nodosa based on the 1990 ACR criteria using the 2007 EMA algorithm modified by the 2012 CHCC definitions. ( 28980907 )
2017
50
Sudden Death from Spontaneous Coronary Artery Dissection due to Polyarteritis Nodosa. ( 29209584 )
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 acute-phase response GO:0006953 9.16 CRP IL6
2 inflammatory response GO:0006954 9.02 CRP IFNA2 IL6 MEFV SELE
3 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 heparin binding GO:0008201 8.8 ADA2 APOH MPO

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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