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 72 49 55 36 51 41 14 69
Periarteritis 49 69
Polyarteritis 49 69
Pan 49 55
Küssmaul-Maier Disease 55
Periarteritis Nodosa 55

Characteristics:

Orphanet epidemiological data:

55
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 32 M30.0 M30.1
ICD9CM 34 446.0
MeSH 41 D010488
NCIt 46 C26847
Orphanet 55 ORPHA767
MESH via Orphanet 42 D010488
UMLS via Orphanet 70 C0031036
ICD10 via Orphanet 33 M30.0
KEGG 36 H01382

Summaries for Polyarteritis Nodosa

NIH Rare Diseases : 49 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 sneddon syndrome and churg-strauss 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 Methotrexate and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and heart, and related phenotype is immune system.

Wikipedia : 72 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 325)
# Related Disease Score Top Affiliating Genes
1 sneddon syndrome 32.2 ADA2 APOH
2 churg-strauss syndrome 31.5 MPO PRTN3
3 kawasaki disease 31.2 CRP IL6 SELE
4 mononeuritis multiplex 30.6 CRP MPO
5 brucellosis 30.6 CRP IL6 MEFV
6 vasculitis 30.4 APOH CRP IFNA2 MEFV MPO PRTN3
7 pyoderma gangrenosum 30.2 IFNA2 MEFV
8 peripheral vascular disease 30.1 CRP IL6 SELE
9 relapsing polychondritis 30.1 CRP IL6
10 adult-onset still's disease 30.0 CRP IL6 MEFV
11 discoid lupus erythematosus 30.0 APOH IFNA2
12 takayasu arteritis 30.0 CRP IL6 SELE
13 pneumonia 30.0 CRP IL6 MPO
14 temporal arteritis 30.0 APOH CRP IL6
15 vascular disease 29.9 APOH CRP SELE
16 palindromic rheumatism 29.9 CRP MEFV PRTN3
17 appendicitis 29.8 CRP IL6 MPO
18 pyoderma 29.7 APOH IFNA2 MEFV
19 rapidly progressive glomerulonephritis 29.7 CRP MPO PRTN3
20 cholangitis 29.7 CRP IL6 PRTN3
21 arteriovenous fistula 29.6 APOH IL6 SELE
22 ulcerative colitis 29.5 CRP IL6 MPO PRTN3
23 acute pancreatitis 29.5 CRP IL6 MPO
24 intermittent claudication 29.4 APOH CRP IL6
25 endocarditis 29.4 APOH CRP IL6
26 wegener granulomatosis 29.1 CRP IL6 MPO PRTN3 SELE
27 inflammatory bowel disease 28.6 CRP IL6 MPO PRTN3 SELE
28 systemic lupus erythematosus 28.1 APOH CRP IL6 MPO PRTN3 SELE
29 polyarteritis nodosa, childhood-onset 12.7
30 cutaneous polyarteritis nodosa 12.5
31 systemic polyarteritis nodosa 12.5
32 primary polyarteritis nodosa 12.4
33 secondary polyarteritis nodosa 12.3
34 single-organ polyarteritis nodosa 12.3
35 retinal vasculitis 11.5
36 autoimmune inner ear disease 11.5
37 adenosine deaminase 2 deficiency 11.5
38 hepatitis 10.7
39 eosinophilic granulomatosis with polyangiitis 10.6
40 hepatitis b 10.6
41 aneurysm 10.5
42 microscopic polyangiitis 10.5
43 relapsing fever 10.4 CRP MEFV
44 amyloidosis aa 10.4 CRP MEFV
45 muckle-wells syndrome 10.4 CRP MEFV
46 chronic orbital inflammation 10.4 IFNA2 PRTN3
47 pharyngitis 10.4 CRP MEFV
48 ethmoid sinusitis 10.4 CRP PRTN3
49 wells syndrome 10.3 CRP MEFV
50 granulomatous hepatitis 10.3 CRP IFNA2

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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.17 CRP IL6 MEFV MPO PECAM1 PRTN3

Drugs & Therapeutics for Polyarteritis Nodosa

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

(show top 50) (show all 59)
# 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 50-18-0, 6055-19-2 2907
3
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
4
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
5
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
6
Prednisone Approved, Vet_approved Phase 4,Phase 2 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
12 Alkylating Agents Phase 4,Phase 2
13 glucocorticoids Phase 4,Phase 2
14 Methylprednisolone acetate Phase 4
15 Methylprednisolone Hemisuccinate Phase 4
16 Folic Acid Antagonists Phase 4,Phase 2
17 Gastrointestinal Agents Phase 4
18 Neuroprotective Agents Phase 4
19 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2
20 Hormone Antagonists Phase 4,Phase 2
21 Dermatologic Agents Phase 4,Phase 2
22 Vitamin B Complex Phase 4,Phase 2
23 Hormones Phase 4,Phase 2
24 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2
25 Peripheral Nervous System Agents Phase 4
26 Antiemetics Phase 4
27 Anti-Infective Agents Phase 4,Phase 2
28 Prednisolone hemisuccinate Phase 4
29 Immunosuppressive Agents Phase 4,Phase 3,Phase 2
30 Prednisolone phosphate Phase 4
31 Antimetabolites Phase 4,Phase 3,Phase 2
32 Anti-Inflammatory Agents Phase 4,Phase 2
33 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2
34 Protective Agents Phase 4
35 Antineoplastic Agents, Hormonal Phase 4,Phase 2
36 Autonomic Agents Phase 4
37 Antirheumatic Agents Phase 4,Phase 3,Phase 2
38 Anti-Bacterial Agents Phase 4,Phase 2
39 Antibiotics, Antitubercular Phase 4
40 Antitubercular Agents Phase 4
41 Folate Nutraceutical Phase 4,Phase 2
42 Vitamin B9 Nutraceutical Phase 4,Phase 2
43
Lamivudine Approved, Investigational Phase 2 134678-17-4 60825
44
Colchicine Approved Phase 2 64-86-8 2833 6167
45
Dapsone Approved, Investigational Phase 2 80-08-0 2955
46 Reverse Transcriptase Inhibitors Phase 2
47 Anti-HIV Agents Phase 2
48 Anti-Retroviral Agents Phase 2
49 Antiviral Agents Phase 2
50 Antimalarials Phase 2

Interventional clinical trials:

(show all 24)

# 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 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 anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone
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 Steroid-Induced Osteoporosis in the Pediatric Population - Canadian Incidence Study Completed NCT01663129
16 Determining Disease Activity Biomarkers in Individuals With Polyarteritis Nodosa Recruiting NCT00315406
17 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis Recruiting NCT01066208
18 Pediatric Vasculitis Initiative Recruiting NCT02006134
19 One-Time DNA Study for Vasculitis Recruiting NCT01241305
20 Journey of Patients With Vasculitis From First Symptom to Diagnosis Recruiting NCT03410290
21 VCRC Tissue Repository Recruiting NCT02967068
22 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
23 Vasculitis Pregnancy Registry Recruiting NCT02593565
24 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:

38
Skin, Kidney, Heart, Eye, Liver, Lung, Bone

Publications for Polyarteritis Nodosa

Articles related to Polyarteritis Nodosa:

(show top 50) (show all 764)
# Title Authors Year
1
Cutaneous polyarteritis nodosa resulting from a paclitaxel-eluting balloon angioplasty. ( 29387747 )
2018
2
Atypical case of classical polyarteritis nodosa: alveolar hemorrhage and positive antineutrophil cytoplasmic antibodya8c. ( 29162217 )
2018
3
Rhabdomyolysis in a Patient with Polyarteritis Nodosa. ( 29021478 )
2018
4
Prostate cancer-associated polyarteritis nodosa: improvement of clinical manifestations after prostatectomy. ( 29352851 )
2018
5
Childhood Polyarteritis Nodosa Presenting With Symmetric Digital Gangrene and Hyperesthesia. ( 29384826 )
2018
6
Correction:<i>Gangrenous digital infarcts in a severe case of cutaneous polyarteritis nodosa</i>. ( 29275379 )
2017
7
Gangrenous digital infarcts in a severe case of cutaneous polyarteritis nodosa. ( 28663247 )
2017
8
Multiple small intestinal perforations in a patient with Hepatitis B Virus-associated Polyarteritis Nodosa. ( 28878877 )
2017
9
The Successful Treatment of Refractory Polyarteritis Nodosa Using Infliximab. ( 28566612 )
2017
10
Asymptomatic cutaneous polyarteritis nodosa: treatment options and therapeutic guidelines. ( 28961286 )
2017
11
Polyarteritis nodosa presenting as peripheral vascular disease and acute limb ischemia. ( 28397742 )
2017
12
Refractory cutaneous polyarteritis nodosa: Successful treatment with etanercept. ( 28626974 )
2017
13
Treatment of polyarteritis nodosa with tocilizumab: a new therapeutic approach? ( 28879047 )
2017
14
Polyarteritis nodosa. ( 28968697 )
2017
15
A Case of Polyarteritis Nodosa Presenting as Rapidly Progressing Intermittent Claudication of Right Leg. ( 29109739 )
2017
16
A case of cutaneous polyarteritis nodosa with PR3-ANCA. ( 28681726 )
2017
17
Polyarteritis Nodosa with a Systemic Inflammatory Response Pattern: Effectiveness of anti-TNF. ( 28372782 )
2017
18
Endovascular coil treatment of a coronary artery aneurysm related to polyarteritis nodosa. ( 29145218 )
2017
19
Sudden Death from Spontaneous Coronary Artery Dissection due to Polyarteritis Nodosa. ( 29209584 )
2017
20
Cytomegalovirus-Induced Polyarteritis Nodosa in a Liver Transplant Recipient. ( 28556523 )
2017
21
Comparing polyarteritis nodosa in children and adults: a single center study. ( 28626961 )
2017
22
Reclassification of polyarteritis nodosa based on the 1990 ACR criteria using the 2007 EMA algorithm modified by the 2012 CHCC definitions. ( 28980907 )
2017
23
Polyarteritis nodosa presenting as peripheral vascular disease and acute limb ischemia. ( 27853042 )
2017
24
The changing face of polyarteritis nodosa and necrotizing vasculitis. ( 28490787 )
2017
25
ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: a multicentre national study. ( 28522451 )
2017
26
Coronary Artery Bypass Grafting in a Patient With Polyarteritis Nodosa. ( 28431718 )
2017
27
Association of five-factor score with the mortality in Japanese patients with polyarteritis nodosa. ( 28648103 )
2017
28
Rare and unusual case of polyarteritis nodosa involving the gastrointestinal tract leading to bowel gangrene. ( 28062430 )
2017
29
The histopathology of cutaneous polyarteritis nodosa and its relationship with lymphocytic thrombophilic arteritis. ( 28198027 )
2017
30
Deficiency of Adenosine Deaminase 2 (DADA2), an Inherited Cause of Polyarteritis Nodosa and a Mimic of Other Systemic Rheumatologic Disorders. ( 28983775 )
2017
31
Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling. ( 28943380 )
2017
32
Acute kidney failure due to polyarteritis nodosa. ( 27939915 )
2017
33
18F-FDG PET/CT Imaging of Popliteal Vasculitis Associated With Polyarteritis Nodosa. ( 28574878 )
2017
34
Inner Peace: Cutaneous Polyarteritis Nodosa. ( 28263733 )
2017
35
Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel. ( 29267446 )
2017
36
Childhood polyarteritis nodosa: diagnosis with non-invasive imaging techniques. ( 27709445 )
2017
37
Chronic polyarthritis as the first manifestation of childhood systemic polyarteritis nodosa. ( 28444097 )
2017
38
Involvement of the Peripheral Nervous System in Polyarteritis Nodosa and Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis. ( 29061248 )
2017
39
Reply to Letter to Editor regarding the article, "Polyarteritis nodosa presenting as peripheral vascular disease and acute limb ischemia". ( 28397743 )
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
Microscopic polyangiitis and non-HBV polyarteritis nodosa with poor-prognosis factors: 10-year results of the prospective CHUSPAN trial. ( 28422001 )
2017
42
Cutaneous polyarteritis nodosa localized to a region of lymphedema secondary to Streptococcus viridans cellulitis and multiple surgeries. ( 27862152 )
2017
43
Optic Neuropathy Secondary to Polyarteritis Nodosa, Case Report, and Diagnostic Challenges. ( 28979236 )
2017
44
Imbalanced expression of dysfunctional regulatory T cells and T-helper cells relates to immunopathogenesis in polyarteritis nodosa. ( 27142840 )
2016
45
Long-term follow-up on tocilizumab treatment of AA amyloidosis secondary to polyarteritis nodosa. ( 27791393 )
2016
46
A case report of cutaneous polyarteritis nodosa in siblings. ( 27299947 )
2016
47
Polyarteritis nodosa presenting as bilateral Wunderlich syndrome: Rare cause of flank pain in a young woman. ( 27865573 )
2016
48
Reappraisal of histopathology of Cutaneous Polyarteritis Nodosa. ( 27592619 )
2016
49
Spontaneous bilateral renal aneurysm rupture secondary to Polyarteritis Nodosa in a patient with chronic myelomonocytic leukaemia: A case report study. ( 27455111 )
2016
50
Intriguing Findings of the Muscle on Magnetic Resonance Imaging in Polyarteritis Nodosa. ( 27803419 )
2016

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 9.28 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 9.62 ADA2 MPO PECAM1 PRTN3
2 acute-phase response GO:0006953 9.4 CRP IL6
3 leukocyte cell-cell adhesion GO:0007159 9.37 PECAM1 SELE
4 negative regulation of lipid storage GO:0010888 9.26 CRP IL6
5 neutrophil extravasation GO:0072672 9.16 PECAM1 PRTN3
6 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.13 IFNA2 IL6 PECAM1
7 inflammatory response GO:0006954 9.02 CRP IFNA2 IL6 MEFV SELE

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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
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47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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