MCID: WGN006
MIFTS: 71

Wegener Granulomatosis

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

Aliases & Classifications for Wegener Granulomatosis

MalaCards integrated aliases for Wegener Granulomatosis:

Name: Wegener Granulomatosis 53 12 49 24 55 13
Granulomatosis with Polyangiitis 53 49 24 55 40 41 69
Wegener's Granulomatosis 12 72 24 36 14
Gpa 49 24 55
Midline Granulomatosis 49 69
Wg 53 49
Pauci-Immune Glomerulonephritis Associated with Granulomatosis with Polyangiitis 69
Necrotizing Respiratory Granulomatosis 12
Granulomatosis - Wegener's 12
Wegeners Granulomatosis 51
Wegener's Syndrome 12

Characteristics:

Orphanet epidemiological data:

55
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 : 40 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 churg-strauss syndrome, 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 Cytokine Signaling in Immune system. The drugs alemtuzumab and Azathioprine have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and kidney, and related phenotypes are hematopoietic system and immune 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 : 24 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 : 49 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.  Last updated: 1/14/2018

OMIM : 53 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 : 72 Granulomatosis with polyangiitis (GPA), formerly known as Wegener\'s granulomatosis (WG), is a systemic... 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 201)
# Related Disease Score Top Affiliating Genes
1 anca-associated vasculitis 33.2 CTLA4 MPO PRTN3 SERPINA1
2 churg-strauss syndrome 30.9 MPO PRTN3 TNF
3 microscopic polyangiitis 30.9 MPO TNF
4 polyarteritis nodosa 30.8 CRP MPO PRTN3 SELE
5 takayasu arteritis 30.7 CRP HLA-DPB1 SELE TNF
6 acute respiratory distress syndrome 30.4 CXCL8 ELANE TNF
7 gingivitis 30.3 CTSG CXCL8 ELANE TNF
8 osteomyelitis 30.3 CXCL8 IFNG TNF
9 mononeuritis multiplex 30.3 CRP MPO
10 meningitis 30.2 CRP CXCL8 IFNG TNF
11 arthritis 29.9 CXCL8 IFNG PTPN22 TNF
12 asthma 29.6 CXCL8 ELANE HLA-DPB1 IFNG MPO SELE
13 rheumatoid arthritis 29.2 CD80 CRP CTLA4 CXCL8 HLA-DPB1 IFNG
14 vasculitis 29.0 BPI CRP CTSG ELANE MPO PRTN3
15 eosinophilic granulomatosis with polyangiitis 12.7
16 autoimmune inner ear disease 10.7 MPO TNF
17 arteritic anterior ischemic optic neuropathy 10.7 CRP MPO PRTN3
18 proctitis 10.6 CRP MPO TNF
19 pleural disease 10.6 CRP MPO TNF
20 fungal esophagitis 10.6 CD80 CRP
21 suppurative cholangitis 10.6 CRP MPO TNF
22 chronic beryllium disease 10.6 HLA-DPB1 IFNG TNF
23 central nervous system vasculitis 10.6 CRP IFNG TNF
24 tuberculous peritonitis 10.6 CRP IFNG TNF
25 leukocyte disease 10.6 CRP ELANE TNF
26 hematopoietic stem cell transplantation 10.6 CTLA4 IFNG TNF
27 hypersensitivity reaction type iii disease 10.6 CRP MPO SELE
28 anterior uveitis 10.6 CTLA4 PTPN22 TNF
29 pustulosis palmaris et plantaris 10.6 ELANE SELE TNF
30 paracoccidioidomycosis 10.6 CTLA4 IFNG TNF
31 staphylococcal toxic shock syndrome 10.6 IFNG TNF
32 erysipelas 10.6 ELANE ELN TNF
33 rapidly progressive glomerulonephritis 10.6 CRP ELANE MPO PRTN3
34 typhoid fever 10.6 CRP IFNG TNF
35 middle ear disease 10.6 CXCL8 PRTN3 TNF
36 orofacial granulomatosis 10.6 CCR5 IFNG TNF
37 filariasis 10.6 CTLA4 IFNG TNF
38 chronic active epstein-barr virus infection 10.6 CTLA4 IFNG SELE
39 cardiac rupture 10.6 CRP CXCL8 ELANE
40 cholangitis 10.6 CRP PRTN3 TNF
41 periodontosis 10.6 CXCL8 ELANE MPO
42 cytomegalovirus retinitis 10.6 CCR5 IFNG TNF
43 louse-borne relapsing fever 10.6 CRP CXCL8 TNF
44 beryllium disease 10.6 HLA-DPB1 TNF
45 plasmodium vivax malaria 10.6 IFNG SELE TNF
46 autoimmune myocarditis 10.6 IFNG TNF
47 perinatal necrotizing enterocolitis 10.5 CRP CXCL8 TNF
48 primary bacterial infectious disease 10.5 CRP IFNG TNF
49 mycobacterium abscessus 10.5 CXCL8 SERPINA1 TNF
50 clonorchiasis 10.5 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:

55 31 (show top 50) (show all 67)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 arthralgia 55 31 hallmark (90%) Very frequent (99-80%) HP:0002829
2 fatigue 55 31 hallmark (90%) Very frequent (99-80%) HP:0012378
3 myalgia 55 31 occasional (7.5%) Occasional (29-5%) HP:0003326
4 fever 55 31 hallmark (90%) Very frequent (99-80%) HP:0001945
5 headache 55 31 occasional (7.5%) Occasional (29-5%) HP:0002315
6 seizures 55 31 occasional (7.5%) Occasional (29-5%) HP:0001250
7 hemoptysis 55 31 frequent (33%) Frequent (79-30%) HP:0002105
8 angina pectoris 55 31 occasional (7.5%) Occasional (29-5%) HP:0001681
9 chest pain 55 31 frequent (33%) Frequent (79-30%) HP:0100749
10 abdominal pain 55 31 frequent (33%) Frequent (79-30%) HP:0002027
11 nausea and vomiting 55 31 frequent (33%) Frequent (79-30%) HP:0002017
12 hemiplegia 55 31 occasional (7.5%) Occasional (29-5%) HP:0002301
13 periorbital edema 55 31 frequent (33%) Frequent (79-30%) HP:0100539
14 hypertension 55 31 occasional (7.5%) Occasional (29-5%) HP:0000822
15 respiratory insufficiency 55 31 frequent (33%) Frequent (79-30%) HP:0002093
16 chronic otitis media 55 31 occasional (7.5%) Occasional (29-5%) HP:0000389
17 recurrent respiratory infections 55 31 hallmark (90%) Very frequent (99-80%) HP:0002205
18 sensorineural hearing impairment 55 31 occasional (7.5%) Occasional (29-5%) HP:0000407
19 visual impairment 55 31 occasional (7.5%) Occasional (29-5%) HP:0000505
20 renal insufficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0000083
21 proteinuria 55 31 frequent (33%) Frequent (79-30%) HP:0000093
22 retinopathy 55 31 occasional (7.5%) Occasional (29-5%) HP:0000488
23 cranial nerve paralysis 55 31 occasional (7.5%) Occasional (29-5%) HP:0006824
24 pulmonary fibrosis 55 31 frequent (33%) Frequent (79-30%) HP:0002206
25 arrhythmia 55 31 occasional (7.5%) Occasional (29-5%) HP:0011675
26 sensory neuropathy 55 31 occasional (7.5%) Occasional (29-5%) HP:0000763
27 weight loss 55 31 hallmark (90%) Very frequent (99-80%) HP:0001824
28 sinusitis 55 31 hallmark (90%) Very frequent (99-80%) HP:0000246
29 autoimmunity 55 31 hallmark (90%) Very frequent (99-80%) HP:0002960
30 venous thrombosis 55 31 occasional (7.5%) Occasional (29-5%) HP:0004936
31 skin ulcer 55 31 occasional (7.5%) Occasional (29-5%) HP:0200042
32 pancreatitis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001733
33 cerebral ischemia 55 31 hallmark (90%) Very frequent (99-80%) HP:0002637
34 intestinal obstruction 55 31 occasional (7.5%) Occasional (29-5%) HP:0005214
35 glomerulopathy 55 31 hallmark (90%) Very frequent (99-80%) HP:0100820
36 hematuria 55 31 hallmark (90%) Very frequent (99-80%) HP:0000790
37 diabetes insipidus 55 31 occasional (7.5%) Occasional (29-5%) HP:0000873
38 chronic obstructive pulmonary disease 55 31 frequent (33%) Frequent (79-30%) HP:0006510
39 purpura 55 31 occasional (7.5%) Occasional (29-5%) HP:0000979
40 gastrointestinal hemorrhage 55 31 occasional (7.5%) Occasional (29-5%) HP:0002239
41 epistaxis 55 31 hallmark (90%) Very frequent (99-80%) HP:0000421
42 pericarditis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001701
43 skin rash 55 31 frequent (33%) Frequent (79-30%) HP:0000988
44 meningitis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001287
45 cough 55 31 frequent (33%) Frequent (79-30%) HP:0012735
46 vasculitis 55 31 hallmark (90%) Very frequent (99-80%) HP:0002633
47 recurrent intrapulmonary hemorrhage 55 31 frequent (33%) Frequent (79-30%) HP:0006535
48 proptosis 55 31 occasional (7.5%) Occasional (29-5%) HP:0000520
49 inflammatory abnormality of the eye 55 31 frequent (33%) Frequent (79-30%) HP:0100533
50 papule 55 31 frequent (33%) 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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 CCR5 CD177 CD80 CTLA4 CTSG ELANE
2 immune system MP:0005387 9.8 CD80 CRP CTLA4 CTSG ELANE IFNG
3 liver/biliary system MP:0005370 9.23 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 113)
# 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 58-05-9 143 6006
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-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1
25 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1
26 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3
27 Antitubercular Agents Phase 4,Phase 3,Phase 2,Phase 1
28 Autonomic Agents Phase 4,Phase 2,Phase 3
29 Dermatologic Agents Phase 4,Phase 3,Phase 2
30 Folic Acid Antagonists Phase 4,Phase 3,Phase 2
31 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1
32 glucocorticoids Phase 4,Phase 2,Phase 3
33 Hormone Antagonists Phase 4,Phase 2,Phase 3
34 Hormones Phase 4,Phase 2,Phase 3,Phase 1
35 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3
36 Methylprednisolone acetate Phase 4,Phase 2,Phase 3
37 Methylprednisolone Hemisuccinate Phase 4,Phase 2,Phase 3
38 Neuroprotective Agents Phase 4,Phase 2,Phase 3
39 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2
40 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1
41 Prednisolone acetate Phase 4,Phase 2,Phase 3
42 Prednisolone hemisuccinate Phase 4,Phase 2,Phase 3
43 Prednisolone phosphate Phase 4,Phase 2,Phase 3
44 Protective Agents Phase 4,Phase 2,Phase 3
45 Vitamin B Complex Phase 4,Phase 3,Phase 2
46 Tin Fluorides Phase 4
47 Folate Nutraceutical Phase 4,Phase 3,Phase 2
48 Vitamin B9 Nutraceutical Phase 4,Phase 3,Phase 2
49 Omega 3 Fatty Acid Nutraceutical Phase 4
50
Etanercept Approved, Investigational Phase 2, Phase 3,Phase 1 185243-69-0

Interventional clinical trials:

(show top 50) (show all 118)

# 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 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Completed NCT00307671 Phase 4 prednisone, methylprednisolone,cyclophosphamides;Cyclophosphamide, Azathioprine,prednisone,methylprednisolone;Mycophenolate mofetil,methotrexate
4 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Recruiting NCT02626845 Phase 4 Rituximab
5 Low-dose Glucocorticoid Vasculitis Induction Study Recruiting NCT02198248 Phase 4 Rituximab;Glucocorticoids
6 Pilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis Active, not recruiting NCT02169219 Phase 4 Glucocorticoids;Rituximab
7 Maintenance of Remission With Rituximab Versus Azathioprine for Newly-diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. Not yet recruiting NCT03164473 Phase 4 Rituximab;Azathioprine;Placebo-rituximab;Placebo-azathioprine
8 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
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 Comparison Study of Two Rituximab Regimens in the Remission of ANCA Associated Vasculitis Completed NCT01731561 Phase 3 Rituximab (Arm B);Rituximab (Arm A)
23 Prevention of Glucocorticoid-Induced Osteoporosis in Rheumatic Diseases: Alendronate Versus Alfacalcidol. Completed NCT00138983 Phase 3 Alendronate versus alfacalcidol (1-alpha OH vitamin D)
24 Rituximab in Eosinophilic Granulomatosis With Polyangiitis Recruiting NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
25 Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) Recruiting NCT02108860 Phase 3 Abatacept;placebo
26 The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach Recruiting NCT01933724 Phase 3 5 mg prednisone;0 mg prednisone
27 The Assessment of Prednisone In Remission Trial - Centers of Excellence Approach Recruiting NCT01940094 Phase 3 Prednisone 5 mg/day;Prednisone 0 mg/day
28 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
29 ASCENT-Study of Sacituzumab Govitecan in Refractory/Relapsed Triple-Negative Breast Cancer Recruiting NCT02574455 Phase 3 Sacituzumab govitecan;Eribulin;Capecitabine;Gemcitabine;Vinorelbine
30 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
31 Long-term Access Program (LAP) of Mepolizumab for Subjects Who Participated in Study MEA115921 Active, not recruiting NCT03298061 Phase 3 Mepolizumab;Prednisolone
32 Rituximab Vasculitis Maintenance Study Active, not recruiting NCT01697267 Phase 3 Azathioprine
33 Plasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis Active, not recruiting NCT00987389 Phase 3 Glucocorticoids
34 Comparison Between a Long Term and a Conventional Maintenance Treatment With Rituximab Active, not recruiting NCT02433522 Phase 3 rituximab;Placebo
35 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
36 Intravenous Immunoglobulin After Relapse in Vasculitis Terminated NCT00307658 Phase 3 Intravenous immunoglobulins (human immunoglobulins G)
37 Plasma Exchange for Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3 Intravenous methyl prednisolone;Methyl prednisolone
38 IMPROVE: Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis Terminated NCT00307645 Phase 3 Cyclophosphamide;Mycophenolate mofetil;Azathioprine;Prednisone (and methylprednisolone)
39 Phase 3 Trial of 90Y-Clivatuzumab Tetraxetan & Gemcitabine vs Placebo & Gemcitabine in Metastatic Pancreatic Cancer Terminated NCT01956812 Phase 3 IMMU-107;placebo;Gemcitabine
40 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
41 Phase Ib Study of SC Milatuzumab in SLE Unknown status NCT01845740 Phase 1, Phase 2 milatuzumab;Placebo
42 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
43 Study of Veltuzumab (hA20) at Different Doses in Patients With ITP Unknown status NCT00547066 Phase 1, Phase 2
44 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
45 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2 Gusperimus
46 Abatacept in Treating Adults With Mild Relapsing Wegener's Granulomatosis Completed NCT00468208 Phase 1, Phase 2 Abatacept
47 Daclizumab to Treat Wegener's Granulomatosis Completed NCT00040248 Phase 2 Daclizumab
48 Etanercept to Treat Wegener's Granulomatosis Completed NCT00001901 Phase 2 Etanercept
49 Treatment of Wegener's Granulomatosis With Cyclophosphamide Completed NCT00001155 Phase 2 cyclophosphamide
50 Comparison of Treatments to Maintain Disease Remission in Patients With Wegener's Granulomatosis and Related Vasculitis Syndromes Completed NCT00004567 Phase 2 Mycophenolate Mofetil

Search NIH Clinical Center for Wegener Granulomatosis

Inferred drug relations via UMLS 69 / NDF-RT 47 :


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:

18
Lung, Skin, Kidney

MalaCards organs/tissues related to Wegener Granulomatosis:

38
Lung, Kidney, Skin, Testes, Trachea, Neutrophil, Monocytes

Publications for Wegener Granulomatosis

Articles related to Wegener Granulomatosis:

(show top 50) (show all 473)
# Title Authors Year
1
Eosinophilic granulomatosis with polyangiitis: A case report. ( 29428428 )
2018
2
Granulomatosis With Polyangiitis Associated With Mumps Viral Infection. ( 29424761 )
2018
3
Orbital granulomatosis with polyangiitis masquerading as invasive fungal sinusitis. ( 29303390 )
2018
4
Multiple Cerebral Infarctions Due to Patent Foramen Ovale in a Patient with Eosinophilic Granulomatosis with Polyangiitis. ( 29373226 )
2018
5
Frequency, Diagnosis, Treatment, and Outcome of Gastrointestinal Disease in Granulomatosis with Polyangiitis and Microscopic Polyangiitis. ( 29419474 )
2018
6
Polyps, grommets and eosinophilic granulomatosis with polyangiitis. ( 29310745 )
2018
7
Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis. ( 29436199 )
2018
8
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
9
Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male. ( 29366363 )
2018
10
Diffuse Alveolar Hemorrhage Developing Immediately after Immunosuppressive Treatments in a Patient with Granulomatosis with Polyangiitis Who Had Pulmonary Nodules. ( 29093401 )
2018
11
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
12
Granulomatosis with Polyangiitis Presenting with Pyoderma Gangrenosum-Like Ulceration and Negative Cytoplasmic Antineutrophilic Cytoplasmic Antibodies in a Child. ( 28884919 )
2017
13
Breast granulomatosis with polyangiitis mimicking breast cancer. ( 29449734 )
2017
14
Granulomatosis with polyangiitis (Wegener granulomatosis): a proteinase-3 driven disease? ( 28495524 )
2017
15
Omalizumab-associated eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). ( 28094120 )
2017
16
Neutrophil extracellular traps formation in patients with eosinophilic granulomatosis with polyangiitis: association with eosinophilic inflammation. ( 28229830 )
2017
17
Recurrent Transitory Ischemic Attacks with Subsequent Development of the Ischemic Stroke as Initial Manifestation of Eosinophilic Granulomatosis with Polyangiitis in Depressed Patient. ( 29197212 )
2017
18
Imaging diagnosis of orbital Wegener granulomatosis: A rare case report. ( 28591026 )
2017
19
Vasculitis: Mepolizumab for eosinophilic granulomatosis with polyangiitis. ( 28769112 )
2017
20
Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis. ( 28251013 )
2017
21
Central nervous system involvement in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Report of 26 patients and review of the literature. ( 28709761 )
2017
22
Interferon-I+ for Induction and Maintenance of Remission in Eosinophilic Granulomatosis with Polyangiitis: A Single-center Retrospective Observational Cohort Study. ( 28412705 )
2017
23
Refractory optic perineuritis due to granulomatosis with polyangiitis successfully treated with methotrexate and mycophenolate mofetil combination therapy. ( 28293459 )
2017
24
Granulomatosis with Polyangiitis (Wegener Granulomatosis) with Unusual Presentation. ( 28260778 )
2017
25
Demographic and Clinical Characteristics Associated with Central Nervous System Hemorrhage in Patients with Eosinophilic Granulomatosis with Polyangiitis: A Case Report and Review of the Literature. ( 28864668 )
2017
26
Basophil and M2 macrophage infiltration in lesional skin of eosinophilic granulomatosis with polyangiitis. ( 28943503 )
2017
27
Eosinophilic granulomatosis with polyangiitis complicated by subarachnoid hemorrhage and coronary vasculitis: a case report and review of the literature. ( 29127573 )
2017
28
Neurological Complications in Eosinophilic Granulomatosis with Polyangiitis (EGPA): The Roles of History and the Results of Physical Examinations in the Diagnosis of EGPA. ( 28924115 )
2017
29
Classification of eosinophilic granulomatosis with polyangiitis phenotypes and treatment based on phenotypes. ( 28711579 )
2017
30
A Case of Churg-Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis (EGPA)) of the Urinary Bladder. ( 28477943 )
2017
31
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. ( 28514601 )
2017
32
Lesson of the month 1: Beware the atypical presentation: eosinophilic granulomatosis with polyangiitis presenting as acute coronary syndrome. ( 28365634 )
2017
33
Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). ( 27671089 )
2017
34
Longterm Prognosis of 121 Patients with Eosinophilic Granulomatosis with Polyangiitis in Japan. ( 28572468 )
2017
35
IgA antibodies to myeloperoxidase in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss). ( 28281453 )
2017
36
Rituximab as Induction Therapy in Eosinophilic Granulomatosis with Polyangiitis Refractory to Conventional Immunosuppressive Treatment: A 36-Month Follow-Up Analysis. ( 28916432 )
2017
37
Five factor score more than 1 is associated with relapse during the first 2 year-follow up in patients with eosinophilic granulomatosis with polyangiitis. ( 28261989 )
2017
38
Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse: A case report. ( 28353556 )
2017
39
Successful treatment with hyperbaric oxygen therapy for pneumatosis cystoides intestinalis as a complication of granulomatosis with polyangiitis: a case report. ( 28917259 )
2017
40
Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. ( 28905856 )
2017
41
Heart failure not responsive to standard immunosuppressive therapy is successfully treated with high dose intravenous immunoglobulin therapy in a patient with Eosinophilic Granulomatosis with Polyangiitis (EGPA). ( 28152445 )
2017
42
Colitis as a form of presentation of eosinophilic granulomatosis with polyangiitis. ( 28645447 )
2017
43
Eosinophilic granulomatosis with polyangiitis and mononeuritis multiplex responded to induction cyclophosphamide. ( 28551593 )
2017
44
Healing of leg ulcers associated with granulomatosis with polyangiitis (Wegener granulomatosis) after rituximab therapy. ( 28207017 )
2017
45
Immunoglobulin G4-related Chronic Rhinosinusitis: A Pitfall in the Differential Diagnosis of Granulomatosis with Polyangiitis, Rosai-Dorfman Disease and Fungal Rhinosinusitis. ( 29288039 )
2017
46
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Complicated by Perforation of the Small Intestine and Cholecystitis. ( 29269641 )
2017
47
Unusual cardiac involvement in granulomatosis with polyangiitis manifesting as acute congestive heart failure. ( 28766511 )
2017
48
Eosinophilic granulomatosis with polyangiitis - toward personalized treatment. ( 28950436 )
2017
49
Eosinophilic granulomatosis with polyangiitis followed by venous thromboembolism resulting in severe cutaneous ulcers. ( 28556974 )
2017
50
Sinonasal imaging findings in granulomatosis with polyangiitis (Wegener granulomatosis): A systematic review. ( 28234146 )
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 26)
# 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
12.71 CD80 CTLA4 CXCL8 HLA-DPB1 IFNG TNF
4 12.46 CTLA4 IFNG MPO PTPN22 TNF
5
Show member pathways
12.34 CCR5 CTLA4 IFNG TNF
6
Show member pathways
12.28 BPI CTSG ELANE PRTN3
7
Show member pathways
12.23 CRP IFNG MPO TNF
8
Show member pathways
12.16 CCR5 HLA-DPB1 IFNG TNF
9 12.15 CXCL8 ELANE MPO RXRB
10 12 CD80 CTLA4 HLA-DPB1 SELE
11 11.84 CD80 IFNG MPO TNF
12 11.74 CTSG CXCL8 IFNG TNF
13 11.71 CXCL8 IFNG SELE
14 11.67 ELANE MPO PRTN3
15 11.62 CD80 CTLA4 PTPN22
16
Show member pathways
11.59 CTLA4 CXCL8 IFNG TNF
17 11.48 CXCL8 IFNG SELE
18 11.47 CXCL8 ELN SERPINA1 TNF
19 11.42 ELANE MPO TNF
20
Show member pathways
11.42 CXCL8 IFNG SELE TNF
21 11.29 ELANE ELN SELE
22 11.25 CCR5 CD80 CXCL8 TNF
23 11.23 IFNG SELE TNF
24 11.15 CXCL8 IFNG SELE TNF
25 11.05 CD80 CTLA4 CXCL8 HLA-DPB1 IFNG TNF
26 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 9.9 BPI CRP CTSG CXCL8 ELANE ELN
2 cell surface GO:0009986 9.8 CCR5 CD80 CTSG ELANE HLA-DPB1 TNF
3 extracellular space GO:0005615 9.7 BPI CRP CTSG CXCL8 ELANE IFNG
4 secretory granule GO:0030141 9.54 CTSG ELANE MPO
5 protein complex involved in cell adhesion GO:0098636 9.26 CD80 CTLA4
6 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 24)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.93 CCR5 CRP CXCL8 SELE TNF
2 leukocyte migration GO:0050900 9.89 CD177 ELANE SELE TNF
3 defense response GO:0006952 9.81 CCR5 CXCL8 MPO TNF
4 extracellular matrix disassembly GO:0022617 9.79 CTSG ELANE ELN
5 cellular response to lipopolysaccharide GO:0071222 9.78 CCR5 CTSG CXCL8 TNF
6 T cell costimulation GO:0031295 9.77 CD80 CTLA4 HLA-DPB1
7 defense response to bacterium GO:0042742 9.77 BPI CTSG ELANE MPO TNF
8 calcium-mediated signaling GO:0019722 9.72 CCR5 CXCL8 SELE
9 response to lipopolysaccharide GO:0032496 9.72 CTSG ELANE MPO PTPN22 SELE
10 neutrophil degranulation GO:0043312 9.7 BPI CD177 CTSG ELANE MPO PRTN3
11 positive regulation of superoxide anion generation GO:0032930 9.62 CD177 CRP
12 positive regulation of immune response GO:0050778 9.61 CTSG ELANE
13 defense response to fungus GO:0050832 9.61 CTSG ELANE MPO
14 negative regulation of lipid storage GO:0010888 9.58 CRP TNF
15 leukocyte migration involved in inflammatory response GO:0002523 9.58 ELANE SELE
16 cell-cell junction maintenance GO:0045217 9.57 CD177 PRTN3
17 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.56 ELANE TNF
18 antimicrobial humoral response GO:0019730 9.56 BPI CTSG ELANE PRTN3
19 immune response GO:0006955 9.56 BPI CCR5 CTLA4 CTSG CXCL8 HLA-DPB1
20 neutrophil extravasation GO:0072672 9.52 CD177 PRTN3
21 response to yeast GO:0001878 9.49 ELANE MPO
22 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.46 IFNG TNF
23 positive regulation of vitamin D biosynthetic process GO:0060557 9.4 IFNG TNF
24 negative regulation of growth of symbiont in host GO:0044130 8.92 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
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
46 NCIt
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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