WG
MCID: WGN006
MIFTS: 63

Wegener Granulomatosis (WG) malady

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

Aliases & Classifications for Wegener Granulomatosis

Aliases & Descriptions for Wegener Granulomatosis:

Name: Wegener Granulomatosis 54 12 50 25 56 13
Granulomatosis with Polyangiitis 50 25 56 41 42 69
Wegener's Granulomatosis 12 25 14
Gpa 50 25 56
Midline Granulomatosis 50 69
Pauci-Immune Glomerulonephritis Associated with Granulomatosis with Polyangiitis 69
Necrotizing Respiratory Granulomatosis 12
Granulomatosis - Wegener's 12
Wegeners Granulomatosis 52
Wegener's Syndrome 12
Wg 50

Characteristics:

Orphanet epidemiological data:

56
granulomatosis with polyangiitis
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe),1-5/10000 (Sweden),1-9/1000000 (Germany),1-9/100000 (France),1-9/100000 (Sweden),1-9/100000 (Norway),1-9/100000 (Australia),1-9/100000 (New Zealand),1-9/100000 (United States),1-9/100000 (China),1-9/1000000 (Lithuania),1-9/1000000 (Greece),1-9/1000000 (Italy),1-9/1000000 (Canada),1-9/1000000 (Japan),<1/1000000 (Taiwan, Province of China),1-9/100000 (Italy),1-5/10000 (Denmark); Age of onset: All ages; Age of death: any age;

Classifications:



External Ids:

OMIM 54 608710
Disease Ontology 12 DOID:12132
ICD10 33 M31.3 M31.30
ICD9CM 35 446.4
MeSH 42 D014890
NCIt 47 C3444
Orphanet 56 ORPHA900
MESH via Orphanet 43 D014890
UMLS via Orphanet 70 C0043092
ICD10 via Orphanet 34 M31.3
UMLS 69 C3495801

Summaries for Wegener Granulomatosis

OMIM : 54 Wegener granulomatosis (WG) is a systemic disease with a complex genetic background. It is characterized by necrotizing... (608710) more...

MalaCards based summary : Wegener Granulomatosis, also known as granulomatosis with polyangiitis, is related to labyrinthitis and choroiditis, and has symptoms including arthralgia, fatigue and myalgia. An important gene associated with Wegener Granulomatosis is WG (Wegener Granulomatosis), and among its related pathways/superpathways are Innate Immune System and Allograft rejection. The drugs alemtuzumab and Azathioprine have been mentioned in the context of this disorder. Affiliated tissues include lung, kidney and skin, and related phenotypes are hematopoietic system and cardiovascular system

Disease Ontology : 12 An autoimmune disease that is located in lung, located in kidney, located in skin resulting from an autoimmune attack by antineutrophil cytoplasmic antibodies against small and medium-size blood vessels.

Genetics Home Reference : 25 Granulomatosis with polyangiitis (GPA) is a condition that causes inflammation that primarily affects the respiratory tract (including the lungs and airways) and the kidneys. This disorder is also commonly known as Wegener granulomatosis. A characteristic feature of GPA is inflammation of blood vessels (vasculitis), particularly the small- and medium-sized blood vessels in the lungs, nose, sinuses, windpipe, and kidneys, although vessels in any organ can be involved. Polyangiitis refers to the inflammation of multiple types of vessels, such as small arteries and veins. Vasculitis causes scarring and tissue death in the vessels and impedes blood flow to tissues and organs.

NIH Rare Diseases : 50 granulomatosis with polyangiitis (wegener's) is a type of vasculitis, or inflammation of the blood vessels. this limits the flow of blood to important organs, causing damage. it can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs and kidneys. symptoms can vary in nature and severity, and may include sinus pain; discolored or bloody fluid from the nose; nasal ulcers; constant runny nose (rhinorrhea); joint pain; weakness; tiredness; and/or skin lesions. the cause of granulomatosis with polyangiitis is unknown. early treatment is important. most people improve with medicines to slow or stop the inflammation. last updated: 5/25/2015

MedlinePlus : 41 granulomatosis with polyangiitis (gpa), previously known as wegener's granulomatosis, is a rare disease. it is a type of vasculitis, or inflammation of the blood vessels. the inflammation limits the flow of blood to important organs, causing damage. it can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs, and kidneys. the cause of gpa is unknown. it can affect people at any age. men and women are equally affected. it is more common in whites. symptoms may include joint pain, weakness, tiredness, and cold symptoms such as a runny nose that doesn't get better. doctors use blood tests, chest x-rays, and biopsies to diagnose gpa and rule out other causes of the symptoms. early treatment is important. most people improve with medicines to slow or stop the inflammation. nih: national institute of allergy and infectious diseases

Wikipedia : 71 Granulomatosis with polyangiitis (GPA), previously known as Wegener\'s granulomatosis (WG), is a... more...

Related Diseases for Wegener Granulomatosis

Diseases related to Wegener Granulomatosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 229)
id Related Disease Score Top Affiliating Genes
1 labyrinthitis 29.9 CXCL8 IL10 TNF
2 choroiditis 29.9 CRP CXCL8 IL10 TNF
3 blue toe syndrome 29.8 CXCL8 IFNG IL10 TNF
4 pyoderma gangrenosum 29.4 CXCL8 IFNG TNF
5 goodpasture syndrome 29.4 CCR5 CRP CTLA4 CXCL8 IFNG IL10
6 rheumatoid arthritis 29.4 CRP CTLA4 CXCL8 HLA-DPB1 IFNG IL10
7 eosinophilic granulomatosis with polyangiitis 12.5
8 anca-associated vasculitis 11.5
9 pyridoxal 5'-phosphate-dependent epilepsy 10.4 IL10 TNF
10 autosomal dominant charcot-marie-tooth disease type 2 with giant axons 10.4 MPO TNF
11 villous adenoma 10.4 CRP MPO PRTN3
12 hypolipoproteinemia 10.4 CRP MPO PRTN3
13 scalp dermatosis 10.4 CRP MPO TNF
14 hirschsprung disease 7 10.4 ELANE PRTN3 SELE
15 tubular renal disease-cardiomyopathy syndrome 10.4 IL10 TNF
16 severe combined immunodeficiency, t cell-negative, b-cell/natural killer-cell positive 10.4 IFNG IL10 TNF
17 distal trisomy 14q 10.4 HLA-DPB1 IFNG TNF
18 elephantiasis 10.4 IFNG IL10 PRTN3
19 urticaria 10.4 CRP MPO TNF
20 dancing eyes-dancing feet syndrome 10.4 ELANE ELN MPO
21 cyclosporiasis 10.4 IFNG IL10 TNF
22 subacute glomerulonephritis 10.4 CRP ELANE MPO PRTN3
23 neurotic disorder 10.4 IFNG IL10 TNF
24 hypertropia 10.4 CRP IFNG TNF
25 astrocytoma 10.4 IL10 MPO PRTN3 TNF
26 pericholangitis 10.4 CRP MPO TNF
27 low anorectal malformation 10.4 CTLA4 IL10 TNF
28 tetanus 10.4 ELANE ELN TNF
29 conduct disorder 10.4 IFNG IL10 TNF
30 pancreatic signet ring cell adenocarcinoma 10.4 IFNG IL10 TNF
31 pulpitis 10.4 IFNG IL10 TNF
32 tularemia 10.4 IFNG IL10 TNF
33 urethral intrinsic sphincter deficiency 10.4 IFNG SELE TNF
34 ectodermal dysplasia bartalos type 10.4 IFNG IL10 TNF
35 conidiobolomycosis 10.4 CXCL8 IL10 TNF
36 multiple mitochondrial dysfunctions syndrome 10.4 CRP IFNG IL10
37 hereditary alpha tryptasemia syndrome 10.3 IFNG IL10 TNF
38 rickettsialpox 10.3 IFNG IL10 TNF
39 acute myeloid leukemia with recurrent genetic anomaly 10.3 IFNG IL10 TNF
40 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 10.3 CCR5 IFNG TNF
41 angiomatosis, diffuse corticomeningeal, of divry and van bogaert 10.3 CTLA4 MPO SERPINA1 TNF
42 cardiomyopathy diabetes deafness 10.3 CRP CXCL8 ELANE
43 t-cell prolymphocytic leukemia 10.3 CCR5 PTPN22 TNF
44 microcephaly 10.3 IFNG IL10 TNF
45 omsk hemorrhagic fever 10.3 CXCL8 ELANE MPO
46 radial hypoplasia, triphalangeal thumbs and hypospadias 10.3 ELANE SELE TNF
47 benign secondary hypertension 10.3 CCR5 CTSG MPO
48 rosacea 10.3 IFNG IL10 SELE
49 esotropia 10.3 CRP MPO PRTN3 SELE
50 cicatricial lagophthalmos 10.3 CRP CXCL8 TNF

Comorbidity relations with Wegener Granulomatosis via Phenotypic Disease Network (PDN):


Acute Kidney Failure Chronic Kidney Failure
Deficiency Anemia Neutropenia
Respiratory Failure

Graphical network of the top 20 diseases related to Wegener Granulomatosis:



Diseases related to Wegener Granulomatosis

Symptoms & Phenotypes for Wegener Granulomatosis

Clinical features from OMIM:

608710

Human phenotypes related to Wegener Granulomatosis:

56 32 (show top 50) (show all 67)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 arthralgia 56 32 Very frequent (99-80%) HP:0002829
2 fatigue 56 32 Very frequent (99-80%) HP:0012378
3 myalgia 56 32 Occasional (29-5%) HP:0003326
4 fever 56 32 Very frequent (99-80%) HP:0001945
5 headache 56 32 Occasional (29-5%) HP:0002315
6 seizures 56 32 Occasional (29-5%) HP:0001250
7 hemoptysis 56 32 Frequent (79-30%) HP:0002105
8 angina pectoris 56 32 Occasional (29-5%) HP:0001681
9 chest pain 56 32 Frequent (79-30%) HP:0100749
10 abdominal pain 56 32 Frequent (79-30%) HP:0002027
11 nausea and vomiting 56 32 Frequent (79-30%) HP:0002017
12 hemiplegia 56 32 Occasional (29-5%) HP:0002301
13 periorbital edema 56 32 Frequent (79-30%) HP:0100539
14 hypertension 56 32 Occasional (29-5%) HP:0000822
15 respiratory insufficiency 56 32 Frequent (79-30%) HP:0002093
16 chronic otitis media 56 32 Occasional (29-5%) HP:0000389
17 recurrent respiratory infections 56 32 Very frequent (99-80%) HP:0002205
18 sensorineural hearing impairment 56 32 Occasional (29-5%) HP:0000407
19 visual impairment 56 32 Occasional (29-5%) HP:0000505
20 renal insufficiency 56 32 Occasional (29-5%) HP:0000083
21 proteinuria 56 32 Frequent (79-30%) HP:0000093
22 retinopathy 56 32 Occasional (29-5%) HP:0000488
23 cranial nerve paralysis 56 32 Occasional (29-5%) HP:0006824
24 pulmonary fibrosis 56 32 Frequent (79-30%) HP:0002206
25 arrhythmia 56 32 Occasional (29-5%) HP:0011675
26 sensory neuropathy 56 32 Occasional (29-5%) HP:0000763
27 weight loss 56 32 Very frequent (99-80%) HP:0001824
28 sinusitis 56 32 Very frequent (99-80%) HP:0000246
29 autoimmunity 56 32 Very frequent (99-80%) HP:0002960
30 venous thrombosis 56 32 Occasional (29-5%) HP:0004936
31 skin ulcer 56 32 Occasional (29-5%) HP:0200042
32 pancreatitis 56 32 Occasional (29-5%) HP:0001733
33 cerebral ischemia 56 32 Very frequent (99-80%) HP:0002637
34 intestinal obstruction 56 32 Occasional (29-5%) HP:0005214
35 glomerulopathy 56 32 Very frequent (99-80%) HP:0100820
36 hematuria 56 32 Very frequent (99-80%) HP:0000790
37 diabetes insipidus 56 32 Occasional (29-5%) HP:0000873
38 chronic obstructive pulmonary disease 56 32 Frequent (79-30%) HP:0006510
39 purpura 56 32 Occasional (29-5%) HP:0000979
40 gastrointestinal hemorrhage 56 32 Occasional (29-5%) HP:0002239
41 epistaxis 56 32 Very frequent (99-80%) HP:0000421
42 pericarditis 56 32 Occasional (29-5%) HP:0001701
43 skin rash 56 32 Frequent (79-30%) HP:0000988
44 meningitis 56 32 Occasional (29-5%) HP:0001287
45 cough 56 32 Frequent (79-30%) HP:0012735
46 vasculitis 56 32 Very frequent (99-80%) HP:0002633
47 recurrent intrapulmonary hemorrhage 56 32 Frequent (79-30%) HP:0006535
48 proptosis 56 32 Occasional (29-5%) HP:0000520
49 inflammatory abnormality of the eye 56 32 Frequent (79-30%) HP:0100533
50 papule 56 32 Frequent (79-30%) HP:0200034

UMLS symptoms related to Wegener Granulomatosis:


angina pectoris, chest pain, coughing, edema, snoring

MGI Mouse Phenotypes related to Wegener Granulomatosis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 CCR5 CD177 CTLA4 CTSG ELANE IFNG
2 cardiovascular system MP:0005385 9.96 CCR5 CRP CTLA4 IFNG IL10 MPO
3 immune system MP:0005387 9.8 CTSG ELANE IFNG IL10 MPO PRTN3
4 liver/biliary system MP:0005370 9.28 PTPN22 RXRB SELE TNF CCR5 CTLA4

Drugs & Therapeutics for Wegener Granulomatosis

Drugs for Wegener Granulomatosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 108)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
alemtuzumab Approved, Investigational Phase 4 216503-57-0
2
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
3
Cyclophosphamide Approved, Investigational Phase 4,Phase 3,Phase 2 50-18-0, 6055-19-2 2907
4
Methotrexate Approved Phase 4,Phase 3,Phase 2,Phase 1 1959-05-2, 59-05-2 126941
5
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3 83-43-2 6741
6
Mycophenolate mofetil Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 128794-94-5 5281078
7
Mycophenolic acid Approved Phase 4,Phase 3,Phase 2,Phase 1 24280-93-1 446541
8
Prednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3 50-24-8 5755
9
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3 53-03-2 5865
10
rituximab Approved Phase 4,Phase 2,Phase 3,Phase 1 174722-31-7 10201696
11
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2 59-30-3 6037
12
leucovorin Approved, Nutraceutical Phase 4,Phase 3,Phase 2 58-05-9 54575, 6560146 143
13 Antibodies Phase 4,Phase 2,Phase 3,Phase 1
14 Antibodies, Antineutrophil Cytoplasmic Phase 4,Phase 3,Phase 2
15 Antimetabolites Phase 4,Phase 3,Phase 2,Phase 1
16 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2,Phase 1
17 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1
18 Immunoglobulins Phase 4,Phase 2,Phase 3,Phase 1
19 Immunosuppressive Agents Phase 4,Phase 3,Phase 2,Phase 1
20 Alkylating Agents Phase 4,Phase 3,Phase 2
21 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1
22 Antibiotics, Antitubercular Phase 4,Phase 3,Phase 2,Phase 1
23 Antiemetics Phase 4,Phase 2,Phase 3
24 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1
25 Antineoplastic Agents, Alkylating Phase 4,Phase 3,Phase 2
26 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3
27 Autonomic Agents Phase 4,Phase 2,Phase 3
28 Dermatologic Agents Phase 4,Phase 3,Phase 2
29 Folic Acid Antagonists Phase 4,Phase 3,Phase 2
30 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1
31 glucocorticoids Phase 4,Phase 2,Phase 3
32 Hormone Antagonists Phase 4,Phase 2,Phase 3
33 Hormones Phase 4,Phase 2,Phase 3,Phase 1
34 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3
35 Methylprednisolone acetate Phase 4,Phase 2,Phase 3
36 Methylprednisolone Hemisuccinate Phase 4,Phase 2,Phase 3
37 Neuroprotective Agents Phase 4,Phase 2,Phase 3
38 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2
39 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1
40 Prednisolone acetate Phase 4,Phase 2,Phase 3
41 Prednisolone hemisuccinate Phase 4,Phase 2,Phase 3
42 Prednisolone phosphate Phase 4,Phase 2,Phase 3
43 Protective Agents Phase 4,Phase 2,Phase 3
44 Vitamin B Complex Phase 4,Phase 3,Phase 2
45 Tin Fluorides Phase 4
46 Folate Nutraceutical Phase 4,Phase 3,Phase 2
47 Vitamin B9 Nutraceutical Phase 4,Phase 3,Phase 2
48 Omega 3 Fatty Acid Nutraceutical Phase 4
49
Etanercept Approved, Investigational Phase 2, Phase 3,Phase 1 185243-69-0
50
Gemcitabine Approved Phase 3,Phase 1,Phase 2 95058-81-4 60750

Interventional clinical trials:

(show top 50) (show all 114)
id Name Status NCT ID Phase
1 Alemtuzumab for ANCA Associated Refractory Vasculitis Unknown status NCT01405807 Phase 4
2 Prevention of Relapses in Proteinase 3 (PR3)-Anti-neutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis Unknown status NCT00128895 Phase 4
3 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Completed NCT00307671 Phase 4
4 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Recruiting NCT02626845 Phase 4
5 A Study Evaluating the Safety and Efficacy of Rituximab in Combination With Glucocorticoids in Participants With Wegener's Granulomatosis or Microscopic Polyangitis Recruiting NCT02115997 Phase 4
6 Low-dose Glucocorticoid Vasculitis Induction Study Recruiting NCT02198248 Phase 4
7 Maintenance of ANCA Vasculitis Remission by Intermittent Rituximab Dosing Recruiting NCT02749292 Phase 4
8 Pilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis Active, not recruiting NCT02169219 Phase 4
9 Maintenance of Remission With Rituximab Versus Azathioprine for Newly-diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. Not yet recruiting NCT03164473 Phase 4
10 Interventional Cryotherapy for the Eradication of Benign Airway Disease ("ICE the BAD") Terminated NCT00747461 Phase 4
11 Trial to Study the Effects of Supplementary Omega-3 on Serum C-Reactive Protein Levels Terminated NCT00578578 Phase 4
12 Mycophenolate Mofetil for Treatment of Relapses of Wegener's Disease or Microscopic Polyangiitis (MPA) Unknown status NCT00103792 Phase 3
13 WEGENT - Comparison of Methotrexate or Azathioprine as Maintenance Therapy for ANCA-Associated Vasculitides Unknown status NCT00349674 Phase 3
14 Monoclonal Antibodies in Detecting Residual Disease in Patients Who Have Been Treated for Non-Hodgkin's Lymphoma Unknown status NCT00003338 Phase 2, Phase 3
15 Radiolabeled Monoclonal Antibody in the Detection and Staging of Patients With Non-Hodgkin's Lymphoma Unknown status NCT00003337 Phase 3
16 Etanercept for Wegener's Granulomatosis Completed NCT00005007 Phase 2, Phase 3
17 A Study to Investigate Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis Completed NCT02020889 Phase 3
18 Association Corticosteroid/Azathioprine in Microscopic Polyangiitis/ Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome) Completed NCT00647166 Phase 3
19 Rituximab for the Treatment of Wegener's Granulomatosis and Microscopic Polyangiitis Completed NCT00104299 Phase 2, Phase 3
20 Belimumab in Remission of VASculitis Completed NCT01663623 Phase 3
21 Efficacy Study of Two Treatments in the Remission of Vasculitis Completed NCT00748644 Phase 3
22 Pulse Versus Continuous Cyclophosphamide for Induction of Remission in ANCA-Associated Vasculitides Completed NCT00430105 Phase 2, Phase 3
23 Prevention of Glucocorticoid-Induced Osteoporosis in Rheumatic Diseases: Alendronate Versus Alfacalcidol. Completed NCT00138983 Phase 3
24 Rituximab in Eosinophilic Granulomatosis With Polyangiitis Recruiting NCT02807103 Phase 3
25 Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) Recruiting NCT02108860 Phase 3
26 The Assessment of Prednisone In Remission Trial - Centers of Excellence Approach Recruiting NCT01940094 Phase 3
27 Efficacy and Safety Study of Mepolizumab in Subjects With Severe Hypereosinophilic Syndrome (HES) Recruiting NCT02836496 Phase 3
28 Accelerated v's Standard BEP Chemotherapy for Patients With Intermediate and Poor-risk Metastatic Germ Cell Tumours Recruiting NCT02582697 Phase 3
29 Plasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis Active, not recruiting NCT00987389 Phase 3
30 Comparison Study of Two Rituximab Regimens in the Remission of ANCA Associated Vasculitis Active, not recruiting NCT01731561 Phase 3
31 Rituximab Vasculitis Maintenance Study Active, not recruiting NCT01697267 Phase 3
32 Comparison Between a Long Term and a Conventional Maintenance Treatment With Rituximab Active, not recruiting NCT02433522 Phase 3
33 Study of Sacituzumab Govitecan in Refractory/Relapsed Triple-Negative Breast Cancer Not yet recruiting NCT02574455 Phase 3
34 Clinical Study Comparing the New Immunosuppressive Drug Gusperimus With the Conventional Treatment in Wegener's Granulomatosis Terminated NCT01446211 Phase 3
35 Intravenous Immunoglobulin After Relapse in Vasculitis Terminated NCT00307658 Phase 3
36 Plasma Exchange for Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3
37 IMPROVE: Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis Terminated NCT00307645 Phase 3
38 Phase 3 Trial of 90Y-Clivatuzumab Tetraxetan & Gemcitabine vs Placebo & Gemcitabine in Metastatic Pancreatic Cancer Terminated NCT01956812 Phase 3
39 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides Unknown status NCT00751517 Phase 2
40 Improving Outcomes Following Limb Loss: PALS Plus Unknown status NCT00447655 Phase 1, Phase 2
41 Study of Veltuzumab and 90Y-Epratuzumab in Relapsed/Refractory, Aggressive NHL Unknown status NCT01101581 Phase 1, Phase 2
42 ASIS for Enbrel in Plaque Psoriasis Unknown status NCT02112097 Phase 1, Phase 2
43 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2
44 Daclizumab to Treat Wegener's Granulomatosis Completed NCT00040248 Phase 2
45 Abatacept in Treating Adults With Mild Relapsing Wegener's Granulomatosis Completed NCT00468208 Phase 1, Phase 2
46 Etanercept to Treat Wegener's Granulomatosis Completed NCT00001901 Phase 2
47 Treatment of Wegener's Granulomatosis With Cyclophosphamide Completed NCT00001155 Phase 2
48 Comparison of Treatments to Maintain Disease Remission in Patients With Wegener's Granulomatosis and Related Vasculitis Syndromes Completed NCT00004567 Phase 2
49 Steroids and Methotrexate to Treat Systemic Vasculitis Completed NCT00001256 Phase 2
50 Anti-Cytokine Therapy for Vasculitis Completed NCT00753103 Phase 2

Search NIH Clinical Center for Wegener Granulomatosis

Inferred drug relations via UMLS 69 / NDF-RT 48 :


Cochrane evidence based reviews: granulomatosis with polyangiitis

Genetic Tests for Wegener Granulomatosis

Anatomical Context for Wegener Granulomatosis

MalaCards organs/tissues related to Wegener Granulomatosis:

39
Lung, Kidney, Skin, Trachea, Neutrophil, Testes, Monocytes

The Foundational Model of Anatomy Ontology organs/tissues related to Wegener Granulomatosis:

18
Lung, Skin, Kidney

Publications for Wegener Granulomatosis

Articles related to Wegener Granulomatosis:

(show top 50) (show all 62)
id Title Authors Year
1
Sinonasal imaging findings in granulomatosis with polyangiitis (Wegener granulomatosis): A systematic review. ( 28234146 )
2017
2
Granulomatosis with polyangiitis (Wegener granulomatosis): a proteinase-3 driven disease? ( 28495524 )
2017
3
Granulomatosis with Polyangiitis (Wegener Granulomatosis) with Unusual Presentation. ( 28260778 )
2017
4
Healing of leg ulcers associated with granulomatosis with polyangiitis (Wegener granulomatosis) after rituximab therapy. ( 28207017 )
2017
5
Malignant Pyoderma Associated with Granulomatosis with Polyangiitis (Wegener Granulomatosis) as a Unique Indication for Facial Vascularized Composite Allotransplantation: Part I. ( 27219252 )
2016
6
Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up. ( 28393729 )
2016
7
Sellar Wegener Granulomatosis Masquerading as Cabergoline-Resistant Prolactinoma. ( 27521731 )
2016
8
Orbital involvement as the initial presentation of Wegener granulomatosis in a 9-year-old girl: MR imaging findings. ( 27425807 )
2016
9
Management Scheme for Cerebral Wegener Granulomatosis: An Unusual Pseudotumoral Skull Base Pathology. ( 27671887 )
2016
10
Monoclonal B-cell Lymphocytosis in a Patient with Wegener Granulomatosis: A Case Report and Update on 2016 World Health Organization Classification. ( 27625103 )
2016
11
Tracheal Stenosis Because of Wegener Granulomatosis Misdiagnosed as Asthma. ( 27075424 )
2016
12
Image Gallery: Symmetrical necrosis of the breast as an inaugural manifestation of granulomatosis with polyangiitis (Wegener granulomatosis). ( 27790680 )
2016
13
Wegener Granulomatosis: Otologic Manifestation as First Symptom. ( 26157503 )
2015
14
Wegener granulomatosis as possible cause of vertigo: case report and review. ( 26513951 )
2015
15
Renal cell carcinoma metastatic to the orbit in a patient with Wegener granulomatosis. ( 25295684 )
2015
16
Nasal mucosa narrow band imaging in granulomatosis with polyangiitis (Wegener granulomatosis): A preliminary study. ( 25781561 )
2015
17
Airway Surgery in Tracheostomised Patients with Wegener Granulomatosis Leading to Subglottic Stenosis. ( 27366530 )
2015
18
Total nasal skeletal reconstruction disfigured by granulomatosis with polyangitis (wegener granulomatosis). ( 25750847 )
2015
19
Wegener granulomatosis with supraglottic involvement. ( 26433716 )
2015
20
Wegener granulomatosis-associated optic perineuritis. ( 24144064 )
2014
21
Long-term Outcome of Airway Stenosis in Granulomatosis With Polyangiitis (Wegener Granulomatosis): An Observational Study. ( 25321076 )
2014
22
Changes in sinonasal mucosa in Wegener granulomatosis. ( 24616132 )
2014
23
Limited-Form Wegener Granulomatosis Case: Anaesthetic Approach and Literature Review. ( 27366454 )
2014
24
Coexistent pulmonary granulomatosis with polyangiitis (Wegener granulomatosis) and Crohn disease. ( 24525506 )
2014
25
Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings. ( 25076302 )
2014
26
An unusual orbital localization of wegener granulomatosis detected by 18F-FDG PET/CT. ( 23856831 )
2014
27
Ultrasonographic imaging of the peripheral nerves in a Wegener granulomatosis patient with multiple mononeuropathies. ( 24811470 )
2014
28
Neutrophil proteinase 3 and dipeptidyl peptidase I (cathepsin C) as pharmacological targets in granulomatosis with polyangiitis (Wegener granulomatosis). ( 23385856 )
2013
29
Granulomatosis with polyangiitis (Wegener granulomatosis) as a differential diagnosis of sternal osteomyelitis: the challenges in diagnosis. ( 24263148 )
2013
30
Clinical features and outcomes of 37 Argentinean patients with severe granulomatosis with polyangiitis (wegener granulomatosis). ( 23364664 )
2013
31
Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine. ( 24252079 )
2013
32
Pathology quiz case 3: Wegener granulomatosis. ( 23681045 )
2013
33
Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood. ( 23729544 )
2013
34
Kidney involvement in a Wegener granulomatosis case. ( 23897571 )
2012
35
Palpable purpura, gingival ulcerations and joint pain in a 24-year-old man. Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis). ( 22726723 )
2012
36
Wegener granulomatosis--rare case presentation. ( 23777030 )
2012
37
Wegener granulomatosis presenting as refractory otitis media: a case report. ( 22991991 )
2012
38
Early extracorporeal life support as rescue for Wegener granulomatosis with diffuse alveolar hemorrhage and acute respiratory distress syndrome: a case report and literature review. ( 22158275 )
2011
39
Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement. ( 21674413 )
2011
40
Cardiac involvement in granulomatosis with polyangiitis (Wegener granulomatosis). ( 21947937 )
2011
41
Wegener granulomatosis (granulomatosis with polyangiitis): evolving concepts in treatment. ( 21674414 )
2011
42
Granulomatous choroiditis in Wegener granulomatosis. ( 21482884 )
2011
43
EULAR/PRINTO/PRES criteria for Henoch-SchAPnlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation. ( 20388738 )
2010
44
EULAR/PRINTO/PRES criteria for Henoch-SchAPnlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. ( 20413568 )
2010
45
Isolated blue toe syndrome as the initial sign of Wegener granulomatosis. ( 20609674 )
2010
46
Mucocutaneous leishmaniasis masquerading as Wegener granulomatosis. ( 20375822 )
2010
47
Wegener granulomatosis presenting as epididymitis. ( 19376569 )
2009
48
A hydrophobic patch on proteinase 3, the target of autoantibodies in Wegener granulomatosis, mediates membrane binding via NB1 receptors. ( 18854317 )
2008
49
Labyrinthitis related to Wegener granulomatosis: magnetic resonance imaging findings. ( 18097336 )
2008
50
Mononeuritis multiplex as a presenting feature of Wegener granulomatosis: a case report. ( 17115101 )
2007

Variations for Wegener Granulomatosis

Copy number variations for Wegener Granulomatosis from CNVD:

7
id CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 21629 1 159859611 159867782 Copy number FCGR3B Wegener''s granulomatosis
2 21795 1 161592989 161601158 Copy number FCGR3B Wegener''s granulomatosis

Expression for Wegener Granulomatosis

Search GEO for disease gene expression data for Wegener Granulomatosis.

Pathways for Wegener Granulomatosis

Pathways related to Wegener Granulomatosis according to GeneCards Suite gene sharing:

(show all 31)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.63 CCR5 CD177 CRP CTLA4 CTSG CXCL8
2
Show member pathways
12.78 CTLA4 CXCL8 HLA-DPB1 IFNG IL10 TNF
3
Show member pathways
12.53 HLA-DPB1 IFNG IL10 RXRB TNF
4 12.38 CTLA4 IFNG IL10 MPO PTPN22 TNF
5
Show member pathways
12.33 CCR5 CTLA4 IFNG IL10 TNF
6
Show member pathways
12.26 CRP IFNG MPO TNF
7 12.17 CXCL8 ELANE MPO RXRB
8 12.17 HLA-DPB1 IFNG IL10 TNF
9
Show member pathways
12.15 CCR5 HLA-DPB1 IFNG IL10 TNF
10
Show member pathways
12.08 CXCL8 IFNG IL10 TNF
11 11.92 IFNG IL10 MPO TNF
12 11.74 CXCL8 IFNG SELE
13 11.73 IFNG IL10 TNF
14 11.71 ELANE MPO PRTN3
15 11.69 IFNG IL10 TNF
16 11.69 CXCL8 IL10 TNF
17
Show member pathways
11.68 CTLA4 CXCL8 IFNG TNF
18 11.64 CTLA4 IL10 TNF
19 11.64 CTSG CXCL8 IFNG IL10 TNF
20 11.56 CXCL8 ELN SERPINA1 TNF
21
Show member pathways
11.53 CXCL8 IFNG SELE TNF
22 11.52 CXCL8 IFNG SELE
23 11.46 ELANE MPO TNF
24
Show member pathways
11.36 IFNG IL10 TNF
25 11.36 CCR5 CXCL8 IL10 TNF
26 11.34 ELANE ELN SELE
27 11.31 CTLA4 CXCL8 HLA-DPB1 IFNG TNF
28 11.19 IFNG IL10 TNF
29 11.17 IFNG IL10 SELE TNF
30 10.97 CRP CXCL8 TNF
31 10.71 CXCL8 IFNG IL10 SELE TNF

GO Terms for Wegener Granulomatosis

Cellular components related to Wegener Granulomatosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.7 CRP CTSG CXCL8 ELANE ELN IFNG
2 external side of plasma membrane GO:0009897 9.62 CCR5 CTLA4 IFNG TNF
3 azurophil granule lumen GO:0035578 9.46 CTSG ELANE MPO PRTN3
4 secretory granule GO:0030141 9.43 CTSG ELANE MPO
5 extracellular space GO:0005615 9.36 CRP CTSG CXCL8 ELANE IFNG IL10

Biological processes related to Wegener Granulomatosis according to GeneCards Suite gene sharing:

(show all 27)
id Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.93 CD177 CTSG ELANE MPO PRTN3 SERPINA1
2 leukocyte migration GO:0050900 9.9 CD177 ELANE SELE TNF
3 inflammatory response GO:0006954 9.85 CCR5 CRP CXCL8 IL10 SELE TNF
4 defense response to bacterium GO:0042742 9.8 CTSG ELANE IFNG IL10 MPO TNF
5 extracellular matrix disassembly GO:0022617 9.79 CTSG ELANE ELN
6 defense response to Gram-positive bacterium GO:0050830 9.77 CRP CTSG TNF
7 immune response GO:0006955 9.76 CCR5 CTLA4 CTSG CXCL8 HLA-DPB1 IFNG
8 response to lipopolysaccharide GO:0032496 9.73 CTSG ELANE IL10 MPO PTPN22 SELE
9 antimicrobial humoral response GO:0019730 9.71 CTSG ELANE PRTN3
10 calcium-mediated signaling GO:0019722 9.69 CCR5 CXCL8 SELE
11 response to molecule of bacterial origin GO:0002237 9.63 CXCL8 IL10
12 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.63 IL10 TNF
13 leukocyte migration involved in inflammatory response GO:0002523 9.62 ELANE SELE
14 positive regulation of chemokine biosynthetic process GO:0045080 9.61 IFNG TNF
15 positive regulation of immune response GO:0050778 9.61 CTSG ELANE
16 defense response to fungus GO:0050832 9.61 CTSG ELANE MPO
17 negative regulation of lipid storage GO:0010888 9.58 CRP TNF
18 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.58 ELANE TNF
19 endothelial cell apoptotic process GO:0072577 9.57 IL10 TNF
20 positive regulation of MHC class II biosynthetic process GO:0045348 9.56 IFNG IL10
21 response to yeast GO:0001878 9.55 ELANE MPO
22 negative regulation of cytokine secretion involved in immune response GO:0002740 9.52 IL10 TNF
23 receptor biosynthetic process GO:0032800 9.49 IL10 TNF
24 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.48 IFNG TNF
25 cellular response to lipopolysaccharide GO:0071222 9.43 CCR5 CTSG CXCL8 IFNG IL10 TNF
26 positive regulation of vitamin D biosynthetic process GO:0060557 9.4 IFNG TNF
27 negative regulation of growth of symbiont in host GO:0044130 9.1 CTSG ELANE IFNG IL10 MPO TNF

Molecular functions related to Wegener Granulomatosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 8.92 CXCL8 IFNG IL10 TNF

Sources for Wegener Granulomatosis

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
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