MCID: WGN006
MIFTS: 70

Wegener Granulomatosis

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

Aliases & Classifications for Wegener Granulomatosis

MalaCards integrated aliases for Wegener Granulomatosis:

Name: Wegener Granulomatosis 57 12 53 25 59 13
Granulomatosis with Polyangiitis 57 53 25 59 37 43 44 73
Wegener's Granulomatosis 12 76 25 15
Gpa 53 25 59
Midline Granulomatosis 53 73
Wg 57 53
Pauci-Immune Glomerulonephritis Associated with Granulomatosis with Polyangiitis 73
Necrotizing Respiratory Granulomatosis 12
Granulomatosis - Wegener's 12
Wegeners Granulomatosis 55
Wegener's Syndrome 12

Characteristics:

Orphanet epidemiological data:

59
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:



Summaries for Wegener Granulomatosis

MedlinePlus : 43 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

MalaCards based summary : Wegener Granulomatosis, also known as granulomatosis with polyangiitis, is related to anca-associated vasculitis and microscopic polyangiitis, and has symptoms including angina pectoris, chest pain and coughing. An important gene associated with Wegener Granulomatosis is WG (Wegener Granulomatosis), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs rituximab and alemtuzumab have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and kidney, and related phenotypes are hypertension and seizures

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 : 53 Granulomatosis with polyangiitis (GPA) is a type of vasculitis or swelling (inflammation) of the blood vessels. The disease can cause swelling of the blood vessels anywhere in the body but mainly impacts the sinuses, nose, trachea (windpipe), lungs, and kidneys. The swelling can limit the flow of blood to these body parts, causing damage. Symptoms of the disease can include sinus pain, recurrent respiratory infections, joint pain, tiredness (fatigue), and skin lesions. The exact cause of GPA is unknown, but it is a type of autoimmune disease. Diagnosis of GPA can be made with laboratory tests such as a blood test, biopsy of affected areas, and imaging of the lungs. Treatment of GPA often includes medications such as glucocorticoids and immunosuppressants. 

OMIM : 57 Wegener granulomatosis (WG) is a systemic disease with a complex genetic background. It is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis, vasculitis, and the presence of antineutrophil cytoplasmatic autoantibodies (ANCAs) in patient sera. These ANCAs are antibodies to a defined target antigen, proteinase-3 (PR3, PRTN3; 177020), that is present within primary azurophil granules of neutrophils (PMNs) and lysozymes of monocytes. On cytokine priming of PMNs, PR3 translocates to the cell surface, where PR3-ANCAs can interact with their antigens and activate PMNs. PMNs from patients with active WG express PR3 on their surface, produce respiratory burst, and release proteolytic enzymes after activation with PR3-ANCAs. The consequence is a self-sustaining inflammatory process (Jagiello et al., 2004). (608710)

Wikipedia : 76 Granulomatosis with polyangiitis (GPA), formerly known as Wegener\'s granulomatosis (WG), is a long-term... 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 208)
# Related Disease Score Top Affiliating Genes
1 anca-associated vasculitis 33.5 CTLA4 MPO PRTN3 SERPINA1 TNF
2 microscopic polyangiitis 31.6 MPO TNF
3 churg-strauss syndrome 31.5 MPO PRTN3 TNF
4 polyarteritis nodosa 31.3 CRP MPO PRTN3 SELE
5 takayasu arteritis 31.1 CRP HLA-DPB1 SELE TNF
6 mononeuritis multiplex 30.9 CRP MPO
7 acute respiratory distress syndrome 30.9 CXCL8 ELANE TNF
8 meningitis 30.8 CRP CXCL8 IFNG TNF
9 osteomyelitis 30.7 CXCL8 IFNG TNF
10 gingivitis 30.7 CTSG CXCL8 ELANE TNF
11 arthritis 30.2 CXCL8 IFNG PTPN22 TNF
12 asthma 29.7 CXCL8 ELANE HLA-DPB1 IFNG MPO SELE
13 rheumatoid arthritis 28.7 CD80 CRP CTLA4 CXCL8 HLA-DPB1 IFNG
14 vasculitis 28.6 BPI CRP CTSG ELANE MPO PRTN3
15 eosinophilic granulomatosis with polyangiitis 12.7
16 autoimmune inner ear disease 11.1 MPO TNF
17 arteritic anterior ischemic optic neuropathy 11.0 CRP MPO PRTN3
18 fungal esophagitis 11.0 CD80 CRP
19 pleural disease 11.0 CRP MPO TNF
20 proctitis 11.0 CRP MPO TNF
21 suppurative cholangitis 11.0 CRP MPO TNF
22 chronic beryllium disease 11.0 HLA-DPB1 IFNG TNF
23 leukocyte disease 11.0 CRP ELANE TNF
24 tuberculous peritonitis 11.0 CRP IFNG TNF
25 hematopoietic stem cell transplantation 11.0 CTLA4 IFNG TNF
26 posterior scleritis 11.0 PRTN3 TNF
27 hypersensitivity reaction type iii disease 11.0 CRP MPO SELE
28 staphylococcal toxic shock syndrome 11.0 IFNG TNF
29 pustulosis palmaris et plantaris 11.0 ELANE SELE TNF
30 cerebral aneurysms 11.0 ELANE ELN MPO
31 paracoccidioidomycosis 10.9 CTLA4 IFNG TNF
32 erysipelas 10.9 ELANE ELN TNF
33 rapidly progressive glomerulonephritis 10.9 CRP ELANE MPO PRTN3
34 autoimmune myocarditis 10.9 IFNG TNF
35 typhoid fever 10.9 CRP IFNG TNF
36 middle ear disease 10.9 CXCL8 PRTN3 TNF
37 filariasis 10.9 CTLA4 IFNG TNF
38 chronic active epstein-barr virus infection 10.9 CTLA4 IFNG SELE
39 cholangitis 10.9 CRP PRTN3 TNF
40 periodontosis 10.9 CXCL8 ELANE MPO
41 cytomegalovirus retinitis 10.9 CCR5 IFNG TNF
42 cardiac rupture 10.9 CRP CXCL8 ELANE
43 louse-borne relapsing fever 10.9 CRP CXCL8 TNF
44 orofacial granulomatosis 10.9 CCR5 IFNG TNF
45 plasmodium vivax malaria 10.9 IFNG SELE TNF
46 primary bacterial infectious disease 10.9 CRP IFNG TNF
47 perinatal necrotizing enterocolitis 10.9 CRP CXCL8 TNF
48 mycobacterium abscessus 10.9 CXCL8 SERPINA1 TNF
49 juvenile rheumatoid arthritis 10.9 CRP HLA-DPB1 TNF
50 clonorchiasis 10.9 CXCL8 IFNG 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:

59 32 (show top 50) (show all 67)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hypertension 59 32 occasional (7.5%) Occasional (29-5%) HP:0000822
2 seizures 59 32 occasional (7.5%) Occasional (29-5%) HP:0001250
3 nausea and vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002017
4 respiratory insufficiency 59 32 frequent (33%) Frequent (79-30%) HP:0002093
5 chronic otitis media 59 32 occasional (7.5%) Occasional (29-5%) HP:0000389
6 recurrent respiratory infections 59 32 hallmark (90%) Very frequent (99-80%) HP:0002205
7 sensorineural hearing impairment 59 32 occasional (7.5%) Occasional (29-5%) HP:0000407
8 visual impairment 59 32 occasional (7.5%) Occasional (29-5%) HP:0000505
9 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
10 proteinuria 59 32 frequent (33%) Frequent (79-30%) HP:0000093
11 retinopathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0000488
12 fever 59 32 hallmark (90%) Very frequent (99-80%) HP:0001945
13 cranial nerve paralysis 59 32 occasional (7.5%) Occasional (29-5%) HP:0006824
14 fatigue 59 32 hallmark (90%) Very frequent (99-80%) HP:0012378
15 pulmonary fibrosis 59 32 frequent (33%) Frequent (79-30%) HP:0002206
16 arthralgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002829
17 arrhythmia 59 32 occasional (7.5%) Occasional (29-5%) HP:0011675
18 angina pectoris 59 32 occasional (7.5%) Occasional (29-5%) HP:0001681
19 abdominal pain 59 32 frequent (33%) Frequent (79-30%) HP:0002027
20 sensory neuropathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0000763
21 weight loss 59 32 hallmark (90%) Very frequent (99-80%) HP:0001824
22 sinusitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000246
23 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
24 venous thrombosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0004936
25 skin ulcer 59 32 occasional (7.5%) Occasional (29-5%) HP:0200042
26 pancreatitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001733
27 cerebral ischemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002637
28 myalgia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003326
29 intestinal obstruction 59 32 occasional (7.5%) Occasional (29-5%) HP:0005214
30 glomerulopathy 59 32 hallmark (90%) Very frequent (99-80%) HP:0100820
31 hematuria 59 32 hallmark (90%) Very frequent (99-80%) HP:0000790
32 diabetes insipidus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000873
33 chronic obstructive pulmonary disease 59 32 frequent (33%) Frequent (79-30%) HP:0006510
34 hemoptysis 59 32 frequent (33%) Frequent (79-30%) HP:0002105
35 purpura 59 32 occasional (7.5%) Occasional (29-5%) HP:0000979
36 gastrointestinal hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0002239
37 chest pain 59 32 frequent (33%) Frequent (79-30%) HP:0100749
38 epistaxis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000421
39 pericarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001701
40 skin rash 59 32 frequent (33%) Frequent (79-30%) HP:0000988
41 meningitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001287
42 cough 59 32 frequent (33%) Frequent (79-30%) HP:0012735
43 vasculitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0002633
44 recurrent intrapulmonary hemorrhage 59 32 frequent (33%) Frequent (79-30%) HP:0006535
45 periorbital edema 59 32 frequent (33%) Frequent (79-30%) HP:0100539
46 proptosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000520
47 headache 59 32 occasional (7.5%) Occasional (29-5%) HP:0002315
48 inflammatory abnormality of the eye 59 32 frequent (33%) Frequent (79-30%) HP:0100533
49 papule 59 32 frequent (33%) Frequent (79-30%) HP:0200034
50 elevated c-reactive protein level 59 32 frequent (33%) Frequent (79-30%) HP:0011227

UMLS symptoms related to Wegener Granulomatosis:


angina pectoris, chest pain, coughing, edema, snoring

MGI Mouse Phenotypes related to Wegener Granulomatosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 CD177 CD80 CTLA4 CTSG ELANE IFNG
2 immune system MP:0005387 9.8 CCR5 CD177 CD80 CRP CTLA4 CTSG
3 liver/biliary system MP:0005370 9.23 CCR5 CTLA4 CTSG IFNG PTPN22 RXRB

Drugs & Therapeutics for Wegener Granulomatosis

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

(show top 50) (show all 115)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 174722-31-7 10201696
2
alemtuzumab Approved, Investigational Phase 4 216503-57-0
3
Methotrexate Approved Phase 4,Phase 3,Phase 2,Phase 1 1959-05-2, 59-05-2 126941
4
Cyclophosphamide Approved, Investigational Phase 4,Phase 3,Phase 2,Not Applicable 50-18-0, 6055-19-2 2907
5
Prednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 50-24-8 5755
6
Mycophenolic acid Approved Phase 4,Phase 3,Phase 2,Phase 1 24280-93-1 446541
7
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
8
Mycophenolate mofetil Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 128794-94-5 5281078
9
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 83-43-2 6741
10
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 53-03-2 5865
11
leucovorin Approved, Nutraceutical Phase 4,Phase 3,Phase 2 58-05-9 143 6006
12
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2 59-30-3 6037
13 Prednisolone acetate Phase 4,Phase 2,Phase 3,Not Applicable
14 Alkylating Agents Phase 4,Phase 3,Phase 2,Not Applicable
15 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
16 Methylprednisolone acetate Phase 4,Phase 2,Phase 3,Not Applicable
17 Methylprednisolone Hemisuccinate Phase 4,Phase 2,Phase 3,Not Applicable
18 Folic Acid Antagonists Phase 4,Phase 3,Phase 2
19 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
20 Neuroprotective Agents Phase 4,Phase 2,Phase 3,Not Applicable
21 Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
22 Dermatologic Agents Phase 4,Phase 3,Phase 2,Not Applicable
23 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2
24 Hormones Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
25 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
26 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
27 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1
28 Vitamin B Complex Phase 4,Phase 3,Phase 2
29 Antibiotics, Antitubercular Phase 4,Phase 3,Phase 2,Phase 1
30 Antibodies Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
31 Immunoglobulins Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
32 Antibodies, Antineutrophil Cytoplasmic Phase 4,Phase 3,Phase 2,Not Applicable
33 Immunosuppressive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
34 Prednisolone hemisuccinate Phase 4,Phase 2,Phase 3,Not Applicable
35 Prednisolone phosphate Phase 4,Phase 2,Phase 3,Not Applicable
36 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
37 Antiemetics Phase 4,Phase 2,Phase 3,Not Applicable
38 Protective Agents Phase 4,Phase 2,Phase 3,Not Applicable
39 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
40 Antitubercular Agents Phase 4,Phase 3,Phase 2,Phase 1
41 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
42 Antimetabolites Phase 4,Phase 3,Phase 2,Phase 1
43 Autonomic Agents Phase 4,Phase 2,Phase 3,Not Applicable
44 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2,Phase 1
45 Antineoplastic Agents, Alkylating Phase 4,Phase 3,Phase 2,Not Applicable
46 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
47 Tin Fluorides Phase 4
48 Folate Nutraceutical Phase 4,Phase 3,Phase 2
49 Vitamin B9 Nutraceutical Phase 4,Phase 3,Phase 2
50 Omega 3 Fatty Acid Nutraceutical Phase 4

Interventional clinical trials:

(show top 50) (show all 124)
# Name Status NCT ID Phase Drugs
1 Alemtuzumab for ANCA Associated Refractory Vasculitis Unknown status NCT01405807 Phase 4 Alemtuzumab
2 Prevention of Relapses in Proteinase 3 (PR3)-Anti-neutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis Unknown status NCT00128895 Phase 4 azathioprine
3 Pilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis Completed NCT02169219 Phase 4 Glucocorticoids;Rituximab
4 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Completed NCT00307671 Phase 4 prednisone, methylprednisolone,cyclophosphamides;Cyclophosphamide, Azathioprine,prednisone,methylprednisolone;Mycophenolate mofetil,methotrexate
5 Maintenance of Remission With Rituximab Versus Azathioprine for Newly-diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. Recruiting NCT03164473 Phase 4 Rituximab;Azathioprine;Placebo-rituximab;Placebo-azathioprine
6 Low-dose Glucocorticoid Vasculitis Induction Study Recruiting NCT02198248 Phase 4 Rituximab;Glucocorticoids
7 A Study Evaluating the Safety and Efficacy of Rituximab in Combination With Glucocorticoids in Participants With Wegener's Granulomatosis or Microscopic Polyangitis Suspended NCT02115997 Phase 4 Methylprednisolone;Prednisone;Rituximab
8 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Terminated NCT02626845 Phase 4 Rituximab
9 Interventional Cryotherapy for the Eradication of Benign Airway Disease ("ICE the BAD") Terminated NCT00747461 Phase 4
10 Trial to Study the Effects of Supplementary Omega-3 on Serum C-Reactive Protein Levels Terminated NCT00578578 Phase 4
11 Mycophenolate Mofetil for Treatment of Relapses of Wegener's Disease or Microscopic Polyangiitis (MPA) Unknown status NCT00103792 Phase 3 mycophenolate mofetil;cyclophosphamide
12 WEGENT - Comparison of Methotrexate or Azathioprine as Maintenance Therapy for ANCA-Associated Vasculitides Unknown status NCT00349674 Phase 3 Azathioprine: 2 mg/kg/day;methotrexate 0.3 mg/kg/week, to a maximum and optimal dose of 25 mg/week
13 Monoclonal Antibodies in Detecting Residual Disease in Patients Who Have Been Treated for Non-Hodgkin's Lymphoma Unknown status NCT00003338 Phase 2, Phase 3
14 Radiolabeled Monoclonal Antibody in the Detection and Staging of Patients With Non-Hodgkin's Lymphoma Unknown status NCT00003337 Phase 3
15 Etanercept for Wegener's Granulomatosis Completed NCT00005007 Phase 2, Phase 3 Etanercept
16 Rituximab for the Treatment of Wegener's Granulomatosis and Microscopic Polyangiitis Completed NCT00104299 Phase 2, Phase 3 Rituximab plus cyclophosphamide placebo (rituximab group);Cyclophosphamide plus rituximab placebo (control group);Azathioprine;Methylprednisolone (or other glucocorticoid);Prednisone
17 A Study to Investigate Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis Completed NCT02020889 Phase 3 Placebo
18 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
19 Belimumab in Remission of VASculitis Completed NCT01663623 Phase 3 Azathioprine
20 Efficacy Study of Two Treatments in the Remission of Vasculitis Completed NCT00748644 Phase 3 Rituximab;Azathioprine
21 Pulse Versus Continuous Cyclophosphamide for Induction of Remission in ANCA-Associated Vasculitides Completed NCT00430105 Phase 2, Phase 3 cyclophosphamide
22 Plasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis Completed NCT00987389 Phase 3 Glucocorticoids
23 Comparison Study of Two Rituximab Regimens in the Remission of ANCA Associated Vasculitis Completed NCT01731561 Phase 3 Rituximab (Arm B);Rituximab (Arm A)
24 Prevention of Glucocorticoid-Induced Osteoporosis in Rheumatic Diseases: Alendronate Versus Alfacalcidol. Completed NCT00138983 Phase 3 Alendronate versus alfacalcidol (1-alpha OH vitamin D)
25 Rituximab in Eosinophilic Granulomatosis With Polyangiitis Recruiting NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
26 Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) Recruiting NCT02108860 Phase 3 Abatacept;placebo
27 The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach Recruiting NCT01933724 Phase 3 5 mg prednisone;0 mg prednisone
28 The Assessment of Prednisone In Remission Trial - Centers of Excellence Approach Recruiting NCT01940094 Phase 3 5 mg Prednisone;0 mg Prednisone
29 Efficacy and Safety Study of Mepolizumab in Subjects With Severe Hypereosinophilic Syndrome (HES) Recruiting NCT02836496 Phase 3 Mepolizumab 300 mg;Placebo matching mepolizumab;Active OCS capsules (5 mg prednisolone or prednisone);Placebo matching OCS capsules
30 ASCENT-Study of Sacituzumab Govitecan in Refractory/Relapsed Triple-Negative Breast Cancer Recruiting NCT02574455 Phase 3 Sacituzumab govitecan;Eribulin;Capecitabine;Gemcitabine;Vinorelbine
31 Accelerated v's Standard BEP Chemotherapy for Patients With Intermediate and Poor-risk Metastatic Germ Cell Tumours Recruiting NCT02582697 Phase 3 Bleomycin (active name: Bleomycin Sulfate);Etoposide;Cisplatin;Pegylated G-CSF (Pegfilgrastim);Filgrastim
32 Long-term Access Program (LAP) of Mepolizumab for Subjects Who Participated in Study MEA115921 Active, not recruiting NCT03298061 Phase 3 Mepolizumab;Prednisolone
33 Rituximab Vasculitis Maintenance Study Active, not recruiting NCT01697267 Phase 3 Azathioprine
34 Comparison Between a Long Term and a Conventional Maintenance Treatment With Rituximab Active, not recruiting NCT02433522 Phase 3 rituximab;Placebo
35 Cyclophosphamide vs. Infliximab for Refractory Idiopathic Scleritis (CIRIS) Not yet recruiting NCT03088293 Phase 3 Infliximab;Cyclophosphamide
36 Clinical Study Comparing the New Immunosuppressive Drug Gusperimus With the Conventional Treatment in Wegener's Granulomatosis Terminated NCT01446211 Phase 3 Gusperimus + glucocorticoids;cyclophosphamide followed by methotrexate (azathioprine) + glucocorticoids or methotrexate (azathioprine) + glucocorticoids
37 Intravenous Immunoglobulin After Relapse in Vasculitis Terminated NCT00307658 Phase 3 Intravenous immunoglobulins (human immunoglobulins G)
38 Plasma Exchange for Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3 Intravenous methyl prednisolone;Methyl prednisolone
39 IMPROVE: Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis Terminated NCT00307645 Phase 3 Cyclophosphamide;Mycophenolate mofetil;Azathioprine;Prednisone (and methylprednisolone)
40 Phase 3 Trial of 90Y-Clivatuzumab Tetraxetan & Gemcitabine vs Placebo & Gemcitabine in Metastatic Pancreatic Cancer Terminated NCT01956812 Phase 3 IMMU-107;placebo;Gemcitabine
41 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
42 Phase Ib Study of SC Milatuzumab in SLE Unknown status NCT01845740 Phase 1, Phase 2 milatuzumab;Placebo
43 Study of Veltuzumab and 90Y-Epratuzumab in Relapsed/Refractory, Aggressive NHL Unknown status NCT01101581 Phase 1, Phase 2 Veltuzumab and 90Y-Epratuzumab Tetraxetan;90Y-epratuzumab tetraxetan;veltuzumab
44 Study of Veltuzumab (hA20) at Different Doses in Patients With ITP Unknown status NCT00547066 Phase 1, Phase 2
45 ASIS for Enbrel in Plaque Psoriasis Unknown status NCT02112097 Phase 1, Phase 2 Gadolinium;Gadolinium;Gadolinium;Efficacy of Enbrel subcutaneously at Week 12;Efficacy of Enbrel subcutaneously at Week 24;Efficacy of Enbrel subcutaneously at Week 36;Efficacy of Enbrel subdermally at Week 12;Efficacy of Enbrel subdermally at Week 24;Efficacy of Enbrel subdermally at Week 36;PASI 75 n(%) subcutaneously at Week 12;PASI 75 n(%) subcutaneously at Week 24;PASI 75 n(%) subcutaneously at Week 36;PASI 75 n(%) subdermally at Week 12;PASI 75 n(%) subdermally at Week 24;PASI 75 n(%) subdermally at Week 36;Adverse Reactions of Enbrel subcutaneously;Adverse Reactions of Enbrel subdermally at Week 36;Gadolinium;Gadolinium;Gadolinium
46 Abatacept in Treating Adults With Mild Relapsing Wegener's Granulomatosis Completed NCT00468208 Phase 1, Phase 2 Abatacept
47 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2 Gusperimus
48 Daclizumab to Treat Wegener's Granulomatosis Completed NCT00040248 Phase 2 Daclizumab
49 Etanercept to Treat Wegener's Granulomatosis Completed NCT00001901 Phase 2 Etanercept
50 Treatment of Wegener's Granulomatosis With Cyclophosphamide Completed NCT00001155 Phase 2 cyclophosphamide

Search NIH Clinical Center for Wegener Granulomatosis

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Cochrane evidence based reviews: granulomatosis with polyangiitis

Genetic Tests for Wegener Granulomatosis

Anatomical Context for Wegener Granulomatosis

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

19
Lung, Skin, Kidney

MalaCards organs/tissues related to Wegener Granulomatosis:

41
Lung, Kidney, Skin, Neutrophil, Testes, Trachea, Heart

Publications for Wegener Granulomatosis

Articles related to Wegener Granulomatosis:

(show top 50) (show all 564)
# Title Authors Year
1
Orbital granulomatosis with polyangiitis masquerading as invasive fungal sinusitis. ( 29303390 )
2018
2
Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRareAr cohort. ( 29943913 )
2018
3
Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis. ( 29436199 )
2018
4
Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis. Case Report and Review of the Recent Literature. ( 29446067 )
2018
5
Eosinophilic Granulomatosis With Polyangiitis: Newer Therapies. ( 29611001 )
2018
6
Multiple Cerebral Infarctions Due to Patent Foramen Ovale in a Patient with Eosinophilic Granulomatosis with Polyangiitis. ( 29373226 )
2018
7
Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male. ( 29366363 )
2018
8
Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis. ( 29635229 )
2018
9
Costs of Disease Relapses Among Individuals with Granulomatosis, with Polyangiitis, or Microscopic Polyangiitis in the United States. ( 29470835 )
2018
10
Letter: unlikely liver bedfellows-alpha-1 antitrypsin deficiency and granulomatosis with polyangiitis. ( 29939411 )
2018
11
Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis. ( 29908086 )
2018
12
A Case of dilated Cardiomyopathy with Eosinophilic Granulomatosis with Polyangiitis in which Active Myocardial Inflammation was Only Detected by Endomyocardial Biopsy. ( 29709930 )
2018
13
Letter: unlikely liver bedfellows-alpha-1 antitrypsin deficiency and granulomatosis with polyangiitis. Author's reply. ( 29939419 )
2018
14
Can granulomatosis with polyangiitis be diagnosed earlier in primary care? A case-control study. ( 29340693 )
2018
15
Peripheral Ulcerative Keratitis Associated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab. ( 29321417 )
2018
16
Mepolizumab Treatment of Pediatric Eosinophilic Granulomatosis With Polyangiitis. ( 29466544 )
2018
17
Myeloperoxidase-ANCA-positive granulomatosis with polyangiitis is a distinct subset of ANCA-associated vasculitis: A retrospective analysis of 455 patients from a single center in China. ( 29887327 )
2018
18
Comparison of findings on thoracic computed tomography with the severity and duration of bronchial asthma in patients with eosinophilic granulomatosis with polyangiitis. ( 29857992 )
2018
19
Eosinophilic granulomatosis with polyangiitis and laryngeal involvement: review of the literature and a cross-sectional prospective experience. ( 29888684 )
2018
20
Diffuse Alveolar Hemorrhage Developing Immediately after Immunosuppressive Treatments in a Patient with Granulomatosis with Polyangiitis Who Had Pulmonary Nodules. ( 29093401 )
2018
21
Overall Disability Sum Score for Clinical Assessment of Neurological Involvement in Eosinophilic Granulomatosis With Polyangiitis. ( 29652700 )
2018
22
A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs. ( 29560002 )
2018
23
Histopathological Findings and Increased D-Dimer Are Predictive Factors of Systemic Thromboses in Eosinophilic Granulomatosis With Polyangiitis. ( 29979194 )
2018
24
Aortic Aneurysm as a Complication of Granulomatosis with Polyangiitis Successfully Treated with Prednisolone and Cyclophosphamide: A Case Report and Review of the Literature. ( 29971178 )
2018
25
Hypocomplementemia is associated with worse renal survival in ANCA-positive granulomatosis with polyangiitis and microscopic polyangiitis. ( 29621352 )
2018
26
Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature. ( 29393242 )
2018
27
Polyps, grommets and eosinophilic granulomatosis with polyangiitis. ( 29310745 )
2018
28
A cohort study of comorbidity in patients with granulomatosis with polyangiitis. ( 29069513 )
2018
29
Longterm Outcomes of 188 Japanese Patients with Eosinophilic Granulomatosis with Polyangiitis. ( 29907668 )
2018
30
Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review. ( 29766394 )
2018
31
HBsAg-negative and anti-HBc-positive in eosinophilic granulomatosis with polyangiitis: a retrospective pilot study. ( 29754328 )
2018
32
Eosinophilic granulomatosis with polyangiitis: A case report. ( 29428428 )
2018
33
A case of squamous cell carcinoma of the nasal cavity in a patient with granulomatosis with polyangiitis (Wegener granulomatosis). ( 29493730 )
2018
34
Frequency, Diagnosis, Treatment, and Outcome of Gastrointestinal Disease in Granulomatosis with Polyangiitis and Microscopic Polyangiitis. ( 29419474 )
2018
35
Methylprednisolone and plasmapheresis are effective for life-threatening diffuse alveolar hemorrhage and gastrointestinal hemorrhage in granulomatosis with polyangiitis: A case report and literature review. ( 29703056 )
2018
36
Improving Mortality in End-Stage Renal Disease due to Granulomatosis with Polyangiitis from 1995 to 2014. ( 29361200 )
2018
37
Severe localised granulomatosis with polyangiitis (Wegener's granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. ( 29716529 )
2018
38
Granulomatosis With Polyangiitis Associated With Mumps Viral Infection. ( 29424761 )
2018
39
Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis. ( 29637936 )
2018
40
Eosinophilic Granulomatosis with Polyangiitis Manifested by Cholecystitis and Mononeuritis Multiplex: A Case Report. ( 29892153 )
2018
41
Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report. ( 29564565 )
2018
42
Angioimmunoblastic T-cell lymphoma mimicking eosinophilic granulomatosis with polyangiitis (Churg-Strauss). ( 29966769 )
2018
43
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) induced by immune checkpoint inhibitors. ( 29936437 )
2018
44
Development of Hypertrophic Pachymeningitis in a Patient With Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis. ( 29485545 )
2018
45
Intracranial vasculitis in eosinophilic granulomatosis with polyangiitis. ( 29901786 )
2018
46
Clinical Characteristics and Treatment Response of Peripheral Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center. ( 28079316 )
2017
47
Granulomatosis with polyangiitis can cause periaortitis and pericarditis. ( 29026588 )
2017
48
Lesson of the month 1: Beware the atypical presentation: eosinophilic granulomatosis with polyangiitis presenting as acute coronary syndrome. ( 28365634 )
2017
49
Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse: A case report. ( 28353556 )
2017
50
Cardiac involvement as a presenting feature of eosinophilic granulomatosis with polyangiitis in childhood. ( 28339359 )
2017

Variations for Wegener Granulomatosis

Copy number variations for Wegener Granulomatosis from CNVD:

7
# 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 29)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.45 BPI CCR5 CD177 CD80 CRP CTLA4
2
Show member pathways
13.28 CCR5 CD80 CXCL8 HLA-DPB1 IFNG PRTN3
3
Show member pathways
12.77 CD80 CTLA4 CXCL8 HLA-DPB1 IFNG TNF
4 12.45 CTLA4 IFNG MPO PTPN22 TNF
5
Show member pathways
12.36 HLA-DPB1 IFNG RXRB TNF
6
Show member pathways
12.34 CCR5 CTLA4 IFNG TNF
7
Show member pathways
12.26 BPI CTSG ELANE PRTN3
8
Show member pathways
12.22 CRP IFNG MPO TNF
9
Show member pathways
12.16 CCR5 HLA-DPB1 IFNG TNF
10 12.15 CXCL8 ELANE MPO RXRB
11 11.98 CD80 CTLA4 HLA-DPB1 SELE
12 11.84 CD80 IFNG MPO TNF
13 11.81 CXCL8 SELE TNF
14
Show member pathways
11.81 CTSG ELANE TNF
15 11.74 CTSG CXCL8 IFNG TNF
16 11.71 CXCL8 IFNG SELE
17 11.68 ELANE MPO PRTN3
18 11.63 CD80 CTLA4 PTPN22
19
Show member pathways
11.59 CTLA4 CXCL8 IFNG TNF
20 11.48 CXCL8 IFNG SELE
21 11.47 CXCL8 ELN SERPINA1 TNF
22 11.42 ELANE MPO TNF
23
Show member pathways
11.42 CXCL8 IFNG SELE TNF
24 11.28 ELANE ELN SELE
25 11.25 CCR5 CD80 CXCL8 TNF
26 11.21 IFNG SELE TNF
27 11.15 CXCL8 IFNG SELE TNF
28 11.05 CD80 CTLA4 CXCL8 HLA-DPB1 IFNG TNF
29 10.89 CRP CXCL8 TNF

GO Terms for Wegener Granulomatosis

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.93 BPI CD177 CRP CTSG CXCL8 ELANE
2 cell surface GO:0009986 9.8 CCR5 CD80 CTSG ELANE HLA-DPB1 TNF
3 external side of plasma membrane GO:0009897 9.71 CCR5 CD80 CTLA4 TNF
4 extracellular space GO:0005615 9.7 BPI CRP CTSG CXCL8 ELANE IFNG
5 membrane raft GO:0045121 9.67 CD177 PRTN3 SELE TNF
6 protein complex involved in cell adhesion GO:0098636 9.26 CD80 CTLA4
7 azurophil granule lumen GO:0035578 9.02 BPI CTSG ELANE MPO PRTN3

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

(show all 25)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.96 CCR5 CRP CXCL8 SELE TNF
2 cytokine-mediated signaling pathway GO:0019221 9.89 CCR5 CD80 CXCL8 PRTN3 TNF
3 leukocyte migration GO:0050900 9.88 CD177 ELANE SELE TNF
4 defense response GO:0006952 9.83 CCR5 CXCL8 MPO TNF
5 defense response to Gram-positive bacterium GO:0050830 9.8 CRP CTSG TNF
6 defense response to bacterium GO:0042742 9.8 BPI CTSG ELANE MPO TNF
7 neutrophil degranulation GO:0043312 9.8 BPI CD177 CTSG ELANE MPO PRTN3
8 extracellular matrix disassembly GO:0022617 9.79 CTSG ELANE ELN
9 T cell costimulation GO:0031295 9.79 CD80 CTLA4 HLA-DPB1
10 calcium-mediated signaling GO:0019722 9.72 CCR5 CXCL8 SELE
11 cellular response to lipopolysaccharide GO:0071222 9.72 CCR5 CD80 CTSG CXCL8 TNF
12 antimicrobial humoral response GO:0019730 9.67 BPI CTSG ELANE PRTN3
13 defense response to fungus GO:0050832 9.63 CTSG ELANE MPO
14 response to lipopolysaccharide GO:0032496 9.63 CTSG CXCL8 ELANE MPO PTPN22 SELE
15 positive regulation of immune response GO:0050778 9.61 CTSG ELANE
16 negative regulation of lipid storage GO:0010888 9.6 CRP TNF
17 leukocyte migration involved in inflammatory response GO:0002523 9.59 ELANE SELE
18 cell-cell junction maintenance GO:0045217 9.58 CD177 PRTN3
19 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.57 ELANE TNF
20 neutrophil extravasation GO:0072672 9.54 CD177 PRTN3
21 response to yeast GO:0001878 9.51 ELANE MPO
22 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.48 IFNG TNF
23 positive regulation of vitamin D biosynthetic process GO:0060557 9.43 IFNG TNF
24 immune response GO:0006955 9.28 BPI CCR5 CD80 CTLA4 CTSG CXCL8
25 negative regulation of growth of symbiont in host GO:0044130 9.26 CTSG ELANE MPO TNF

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

# Name GO ID Score Top Affiliating Genes
1 protease binding GO:0002020 8.92 CD177 ELANE SERPINA1 TNF

Sources for Wegener Granulomatosis

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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