PAN
MCID: PLY017
MIFTS: 51

Polyarteritis Nodosa (PAN) malady

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

Aliases & Classifications for Polyarteritis Nodosa

Aliases & Descriptions for Polyarteritis Nodosa:

Name: Polyarteritis Nodosa 12 50 56 29 52 42 14 69
Periarteritis 50 69
Polyarteritis 50 69
Pan 50 56
Küssmaul-Maier Disease 56
Periarteritis Nodosa 56

Characteristics:

Orphanet epidemiological data:

56
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 42 D010488
NCIt 47 C26847
Orphanet 56 ORPHA767
MESH via Orphanet 43 D010488
UMLS via Orphanet 70 C0031036
ICD10 via Orphanet 34 M30.0
UMLS 69 C0031036

Summaries for Polyarteritis Nodosa

NIH Rare Diseases : 50 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. last updated: 11/7/2016

MalaCards based summary : Polyarteritis Nodosa, also known as periarteritis, is related to myocardial infarction and ischemic optic neuropathy. 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 Azathioprine and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and heart, and related phenotype is immune system.

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 287)
id Related Disease Score Top Affiliating Genes
1 myocardial infarction 29.8 CRP IL6 MPO SELE
2 ischemic optic neuropathy 29.7 APOH CRP IL6
3 collagenous colitis 29.5 CRP IL6 MPO
4 histiocytoma 29.5 IFNA2 ITGB2 MEFV
5 rheumatoid arthritis 29.0 CRP IL6 ITGB2 PRTN3
6 cutaneous polyarteritis nodosa 12.4
7 polyarteritis nodosa, childhood-onset 12.4
8 systemic polyarteritis nodosa 12.3
9 primary polyarteritis nodosa 12.3
10 secondary polyarteritis nodosa 12.2
11 single-organ polyarteritis nodosa 12.2
12 retinal vasculitis 11.4
13 adenosine deaminase 2 deficiency 11.4
14 autoimmune inner ear disease 11.4
15 sneddon syndrome 11.1
16 churg-strauss syndrome 10.8
17 kawasaki disease 10.8
18 hepatitis 10.6
19 vasculitis 10.5
20 hepatitis b 10.5
21 aneurysm 10.4
22 microscopic polyangiitis 10.4
23 retinal cancer 10.3 CRP MEFV
24 mechanical lagophthalmos 10.2 CRP MEFV
25 prostate adenocarcinoma 10.2 CRP IFNA2
26 primary lacrimal atrophy 10.2 IFNA2 PRTN3
27 anaplastic ganglioglioma 10.2 CRP MEFV
28 coloboma of iris 10.2 CRP IL6
29 cutaneous sclerosis 10.2 CRP IL6
30 shoulder impingement syndrome 10.2 CRP IL6
31 cannabis dependence 10.2 CRP MPO
32 unilateral multicystic dysplastic kidney 10.2 CRP IL6
33 c1q deficiency 10.2 IFNA2 IL6
34 fallopian tube adenomatoid tumor 10.2 CRP MPO
35 neutrophilia, hereditary 10.2 CRP IL6
36 swine influenza 10.2 CRP IL6
37 cinca syndrome 10.2 CRP MEFV
38 cardiac tuberculosis 10.2 CRP IL6
39 parapharyngeal meningioma 10.2 CRP MPO
40 cataract, total congenital 10.2 CRP IL6
41 laryngotracheoesophageal cleft type 2 10.2 CRP IL6
42 may-hegglin anomaly 10.2 ADA2 APOH
43 hirschsprung disease 7 10.2 PRTN3 SELE
44 gastric lymphoma 10.2 CRP IL6
45 refractory plasma cell neoplasm 10.2 CRP PRTN3
46 human immunodeficiency virus infectious disease 10.2 CRP IL6
47 colonic benign neoplasm 10.2 MPO PRTN3
48 tuberculous salpingitis 10.2 CRP MPO
49 secretory diarrhea myopathy and deafness 10.2 CRP IL6
50 hemangioma of spleen 10.1 CRP IL6

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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.17 ITGB2 MEFV MPO PRTN3 SELE CRP

Drugs & Therapeutics for Polyarteritis Nodosa

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

(show top 50) (show all 59)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
2
Cyclophosphamide Approved, Investigational Phase 4,Phase 2 50-18-0, 6055-19-2 2907
3
Methotrexate Approved Phase 4,Phase 2 1959-05-2, 59-05-2 126941
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
5
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
6
Prednisone Approved, Vet_approved Phase 4,Phase 2 53-03-2 5865
7
Mycophenolate mofetil Approved, Investigational Phase 4 128794-94-5 5281078
8
Mycophenolic acid Approved Phase 4 24280-93-1 446541
9
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2 59-30-3 6037
10
leucovorin Approved, Nutraceutical Phase 4,Phase 2 58-05-9 54575, 6560146 143
11 Alkylating Agents Phase 4,Phase 2
12 Antimetabolites Phase 4,Phase 3,Phase 2
13 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2
14 Antineoplastic Agents, Alkylating Phase 4,Phase 2
15 Antirheumatic Agents Phase 4,Phase 3,Phase 2
16 Immunosuppressive Agents Phase 4,Phase 3,Phase 2
17 Antiemetics Phase 4
18 Anti-Inflammatory Agents Phase 4,Phase 2
19 Antineoplastic Agents, Hormonal Phase 4,Phase 2
20 Autonomic Agents Phase 4
21 Dermatologic Agents Phase 4,Phase 2
22 Folic Acid Antagonists Phase 4,Phase 2
23 Gastrointestinal Agents Phase 4
24 glucocorticoids Phase 4,Phase 2
25 Hormone Antagonists Phase 4,Phase 2
26 Hormones Phase 4,Phase 2
27 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2
28 Methylprednisolone acetate Phase 4
29 Methylprednisolone Hemisuccinate Phase 4
30 Neuroprotective Agents Phase 4
31 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2
32 Peripheral Nervous System Agents Phase 4
33 Prednisolone acetate Phase 4
34 Prednisolone hemisuccinate Phase 4
35 Prednisolone phosphate Phase 4
36 Protective Agents Phase 4
37 Vitamin B Complex Phase 4,Phase 2
38 Anti-Bacterial Agents Phase 4,Phase 2
39 Antibiotics, Antitubercular Phase 4
40 Folate Nutraceutical Phase 4,Phase 2
41 Vitamin B9 Nutraceutical Phase 4,Phase 2
42
Lamivudine Approved, Investigational Phase 2 134678-17-4 60825
43
Colchicine Approved Phase 2 64-86-8 6167 2833
44
Dapsone Approved, Investigational Phase 2 80-08-0 2955
45 Anti-Infective Agents Phase 2
46 Anti-HIV Agents Phase 2
47 Anti-Retroviral Agents Phase 2
48 Antiviral Agents Phase 2
49 Reverse Transcriptase Inhibitors Phase 2
50 Antimalarials Phase 2

Interventional clinical trials:

(show all 23)
id Name Status NCT ID Phase
1 CHUSPAN PAN BP Treatment of Polyarteritis Nodosa and Microscopic Polyangiitis Without Poor-Prognosis Factors Unknown status NCT00400075 Phase 4
2 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Completed NCT00307671 Phase 4
3 Association Corticosteroid/Azathioprine in Microscopic Polyangiitis/ Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome) Completed NCT00647166 Phase 3
4 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides Unknown status NCT00751517 Phase 2
5 Lamivudine for Chronic Hepatitis B Completed NCT00001457 Phase 2
6 Steroids and Methotrexate to Treat Systemic Vasculitis Completed NCT00001256 Phase 2
7 A Randomized Multicenter Study for Isolated Skin Vasculitis Recruiting NCT02939573 Phase 2
8 Hematopoietic Stem Cell Support in Vasculitis Terminated NCT00278512 Phase 1
9 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055
10 Educational Needs of Patients With Systemic Vasculitis Completed NCT02190929
11 Cyclophosphamide and Prednisone Followed by Methotrexate To Treat Vasculitides Completed NCT00001473
12 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
13 Vasculitis Illness Perception (VIP) Study Completed NCT02190916
14 Impact of Vasculitis on Employment and Income Completed NCT02476292
15 Determining Disease Activity Biomarkers in Individuals With Polyarteritis Nodosa Recruiting NCT00315406
16 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis Recruiting NCT01066208
17 Pediatric Vasculitis Initiative Recruiting NCT02006134
18 One-Time DNA Study for Vasculitis Recruiting NCT01241305
19 VCRC Tissue Repository Recruiting NCT02967068
20 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
21 Vasculitis Pregnancy Registry Recruiting NCT02593565
22 Steroid-Induced Osteoporosis in the Pediatric Population - Canadian Incidence Study Active, not recruiting NCT01663129
23 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

Genetic tests related to Polyarteritis Nodosa:

id Genetic test Affiliating Genes
1 Polyarteritis Nodosa 29

Anatomical Context for Polyarteritis Nodosa

MalaCards organs/tissues related to Polyarteritis Nodosa:

39
Skin, Kidney, Heart, Eye, Liver, Lung, Bone

Publications for Polyarteritis Nodosa

Articles related to Polyarteritis Nodosa:

(show top 50) (show all 726)
id Title Authors Year
1
Childhood polyarteritis nodosa: diagnosis with non-invasive imaging techniques. ( 27709445 )
2017
2
Polyarteritis nodosa presenting as peripheral vascular disease and acute limb ischemia. ( 27853042 )
2017
3
Rare and unusual case of polyarteritis nodosa involving the gastrointestinal tract leading to bowel gangrene. ( 28062430 )
2017
4
Acute kidney failure due to polyarteritis nodosa. ( 27939915 )
2017
5
Cutaneous polyarteritis nodosa localized to a region of lymphedema secondary to Streptococcus viridans cellulitis and multiple surgeries. ( 27862152 )
2017
6
Leopard skin appearance of cutaneous polyarteritis nodosa on 18Ffluorodeoxyglucose positron emission tomography. ( 27009824 )
2016
7
Tofacitinib for polyarteritis nodosa: a tailored therapy. ( 27558986 )
2016
8
Long-term follow-up on tocilizumab treatment of AA amyloidosis secondary to polyarteritis nodosa. ( 27791393 )
2016
9
Early-onset stroke, polyarteritis nodosa (PAN), and livedo racemosa. ( 27444081 )
2016
10
Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa. ( 26692417 )
2016
11
Hypertension in the liver clinic - polyarteritis nodosa in a patient with hepatitis B. ( 26989675 )
2016
12
Muscle Involvement in Polyarteritis Nodosa: Report of Eight Cases With Characteristic Contrast Enhancement Pattern on MRI. ( 26797367 )
2016
13
Cytomegalovirus pneumonia and pulmonary haemorrhage in a patient with polyarteritis nodosa. ( 27812074 )
2016
14
Case of cutaneous polyarteritis nodosa with clinical and histopathological features similar to those of livedo vasculopathy. ( 27207329 )
2016
15
Polyarteritis nodosa presenting as bilateral Wunderlich syndrome: Rare cause of flank pain in a young woman. ( 27865573 )
2016
16
Reappraisal of histopathology of Cutaneous Polyarteritis Nodosa. ( 27592619 )
2016
17
Comment on "Polyarteritis nodosa: A contemporary overview". ( 27235122 )
2016
18
Focal gall bladder polyarteritis nodosa in a case of cholelithiasis. ( 27728677 )
2016
19
A diagnostic dilemma: lupus nephritis or renal polyarteritis nodosa? ( 27055352 )
2016
20
Reply to comment on "Polyarteritis nodosa: A contemporary overview". ( 27287297 )
2016
21
Imbalanced expression of dysfunctional regulatory T cells and T-helper cells relates to immunopathogenesis in polyarteritis nodosa. ( 27142840 )
2016
22
Polyarteritis nodosa presenting as a bladder outlet obstruction. ( 27842627 )
2016
23
Polyarteritis nodosa: A contemporary overview. ( 26884100 )
2016
24
Diffuse T2 Signal Hyperintensity of the Teres Minor in a Patient With Polyarteritis Nodosa. ( 27611857 )
2016
25
Spontaneous liver rupture as first sign of polyarteritis nodosa. ( 27917267 )
2016
26
An Autopsy Case of Aortic Intimal Sarcoma Initially Diagnosed as Polyarteritis Nodosa. ( 27803418 )
2016
27
Polyarteritis nodosa with a chronic relapsing course. ( 27721981 )
2016
28
A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide. ( 27018080 )
2016
29
Polyarteritis nodosa in north India: clinical manifestations and outcomes. ( 27990777 )
2016
30
Spontaneous bilateral renal aneurysm rupture secondary to Polyarteritis Nodosa in a patient with chronic myelomonocytic leukaemia: A case report study. ( 27455111 )
2016
31
Epidemiological, clinical and laboratory profiles of cutaneous polyarteritis nodosa patients: Report of 22 cases and literature review. ( 26876385 )
2016
32
Interchanging Autoimmunity - Lupus Mastitis Coexisting with Systemic Polyarteritis Nodosa. ( 27057023 )
2016
33
Spontaneous Retroperitoneal Haematoma due to Polyarteritis Nodosa: Report of a Case and Literature Review. ( 26885433 )
2016
34
Intriguing Findings of the Muscle on Magnetic Resonance Imaging in Polyarteritis Nodosa. ( 27803419 )
2016
35
Polyarteritis nodosa. Diagnostic challenges in a patient with cutaneous vasculitis, psoriasis, psoriatic arthritis and pancytopenia: fatal progression after treatment with G-CSF. ( 27123310 )
2016
36
Presence of anti-phosphatidylserine-prothrombin complex antibodies and anti-moesin antibodies in patients with polyarteritis nodosa. ( 27345569 )
2016
37
A case of isolated renal involvement of polyarteritis nodosa successfully treated with steroid monotherapy. ( 27440849 )
2016
38
A case report of cutaneous polyarteritis nodosa in siblings. ( 27299947 )
2016
39
Childhood-onset systemic polyarteritis nodosa and systemic lupus erythematosus: an overlap syndrome? ( 27914603 )
2016
40
Sacroiliitis and Polyarteritis Nodosa in a Patient with Familial Mediterranean Fever. ( 27143975 )
2016
41
The relationship between lymphocytic thrombophilic arteritis and cutaneous polyarteritis nodosa. ( 27646765 )
2016
42
Novel compound heterozygous variants in CECR1 gene associated with childhood onset polyarteritis nodosa and deficiency of ADA2. ( 27069017 )
2016
43
A hereditary clue for the development of cutaneous polyarteritis nodosa in siblings. ( 27785929 )
2016
44
Allogeneic stem cell transplantation in a patient with myelodysplastic syndrome and polyarteritis nodosa: a case report and systematic review. ( 26787416 )
2016
45
Prolonged pyrexia and subtle skin lesions: polyarteritis nodosa. ( 26972245 )
2016
46
Polyarteritis nodosa confined to the breasts associated with polyenthesitis. ( 25800640 )
2015
47
Polyarteritis nodosa: an atypical presentation. ( 25743873 )
2015
48
Ectopic Adipose Tissue with Vasculitis in the Calf Muscle Explaining Systemic Symptoms in Leg-limited Cutaneous Polyarteritis Nodosa. ( 26059212 )
2015
49
Elicitation of Expert Prior Opinion: Application to the MYPAN Trial in Childhood Polyarteritis Nodosa. ( 25822991 )
2015
50
Pancreatic mass as an initial manifestation of polyarteritis nodosa: a case report and review of the literature. ( 25624739 )
2015

Variations for Polyarteritis Nodosa

ClinVar genetic disease variations for Polyarteritis Nodosa:

6
id Gene Variation Type Significance SNP ID Assembly Location
1 ADA2 NM_001282227.1(ADA2): c.1232A> G (p.Tyr411Cys) single nucleotide variant Pathogenic rs376785840 GRCh37 Chromosome 22, 17662794: 17662794
2 ADA2 NM_001282227.1(ADA2): c.200C> A (p.Ala67Asp) single nucleotide variant Pathogenic rs587777240 GRCh37 Chromosome 22, 17688177: 17688177
3 ADA2 NM_001282227.1(ADA2): c.14G> C (p.Gly5Ala) single nucleotide variant Pathogenic rs200930463 GRCh37 Chromosome 22, 17690428: 17690428
4 ADA2 NM_001282227.1(ADA2): c.210C> G (p.His70Gln) single nucleotide variant Pathogenic rs587777241 GRCh37 Chromosome 22, 17688167: 17688167
5 ADA2 NM_001282227.1(ADA2): c.380G> A (p.Arg127Gln) single nucleotide variant Pathogenic rs77563738 GRCh37 Chromosome 22, 17687997: 17687997
6 ADA2 NM_001282227.1(ADA2): c.13G> A (p.Gly5Arg) single nucleotide variant Pathogenic rs202134424 GRCh37 Chromosome 22, 17690429: 17690429
7 ADA2 NM_001282227.1(ADA2): c.626C> T (p.Pro209Leu) single nucleotide variant Pathogenic rs148936893 GRCh38 Chromosome 22, 17203564: 17203564
8 ADA2 NM_001282225.1(ADA2): c.140G> T (p.Gly47Val) single nucleotide variant Pathogenic rs200930463 GRCh37 Chromosome 22, 17690428: 17690428
9 ADA2 NM_001282227.1(ADA2): c.665G> C (p.Trp222Ser) single nucleotide variant Pathogenic rs587777242 GRCh37 Chromosome 22, 17672663: 17672663

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:

id 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:

id Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.62 ADA2 ITGB2 MPO PRTN3
2 response to lipopolysaccharide GO:0032496 9.61 IL6 MPO SELE
3 aging GO:0007568 9.58 IL6 ITGB2 MPO
4 acute-phase response GO:0006953 9.48 CRP IL6
5 positive regulation of tyrosine phosphorylation of Stat3 protein GO:0042517 9.46 IFNA2 IL6
6 leukocyte cell-cell adhesion GO:0007159 9.37 ITGB2 SELE
7 leukocyte migration involved in inflammatory response GO:0002523 9.26 ITGB2 SELE
8 negative regulation of lipid storage GO:0010888 9.16 CRP IL6
9 inflammatory response GO:0006954 9.1 CRP IFNA2 IL6 ITGB2 MEFV SELE
10 response to yeast GO:0001878 8.96 IL6 MPO

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

id 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
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....