MCID: WGN006
MIFTS: 61

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 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:



Summaries for Wegener Granulomatosis

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

MalaCards based summary : Wegener Granulomatosis, also known as granulomatosis with polyangiitis, is related to polyarteritis nodosa and churg-strauss syndrome, and has symptoms including visual impairment, recurrent respiratory infections and headache. 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, kidney and skin, and related phenotypes are hematopoietic system and immune system

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

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.

OMIM : 54
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)

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.

Wikipedia : 72 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 192)
id Related Disease Score Top Affiliating Genes
1 polyarteritis nodosa 31.2 CRP IFNG TNF
2 churg-strauss syndrome 30.7 CXCL8 IFNG SELE TNF
3 choroiditis 30.6 CRP CXCL8 TNF
4 mucocutaneous leishmaniasis 30.4 CXCL8 IFNG TNF
5 blue toe syndrome 30.4 CXCL8 IFNG TNF
6 asthma 29.8 CXCL8 ELANE HLA-DPB1 IFNG SELE TNF
7 goodpasture syndrome 29.6 CCR5 CRP CTLA4 CXCL8 IFNG SELE
8 rheumatoid arthritis 29.3 CD80 CRP CTLA4 CXCL8 HLA-DPB1 IFNG
9 eosinophilic granulomatosis with polyangiitis 12.5
10 anca-associated vasculitis 11.5
11 autoimmune oophoritis 11.1 MPO TNF
12 central nervous system leiomyosarcoma 11.0 CRP MPO TNF
13 glioblastoma multiforme 11.0 MPO PRTN3 TNF
14 hepatocellular adenoma 11.0 CRP MPO PRTN3
15 scrotum melanoma 10.9 CD80 CRP
16 facial dermatosis 10.9 CRP MPO TNF
17 arthus reaction 10.9 CRP MPO TNF
18 pericholangitis 10.9 CRP MPO TNF
19 spastic paraplegia 43, autosomal recessive 10.9 ELANE PRTN3 SELE
20 mosaic trisomy 1 10.9 HLA-DPB1 IFNG TNF
21 psychologic dyspareunia 10.9 CTLA4 IFNG TNF
22 hemophagocytic lymphohistiocytosis 10.9 CTLA4 IFNG TNF
23 benign mammary dysplasia 10.9 CRP ELANE TNF
24 antigen-peptide-transporter 2 deficiency 10.9 CTLA4 PTPN22 TNF
25 pyridoxine deficiency 10.9 ELANE SELE TNF
26 abnormal retinal correspondence 10.9 CTLA4 IFNG TNF
27 sternal cleft 10.9 IFNG TNF
28 migraine with brainstem aura 10.9 MPO TNF
29 tetanus 10.9 ELANE ELN TNF
30 focal embolic glomerulonephritis 10.9 CRP ELANE MPO PRTN3
31 rift valley fever 10.8 CRP IFNG TNF
32 oropharyngeal cancer, childhood 10.8 CCR5 IFNG TNF
33 testicular infarct 10.8 CXCL8 PRTN3 TNF
34 follicular mucinosis 10.8 CXCL8 ELANE MPO
35 chronic graft versus host disease 10.8 CTLA4 IFNG SELE
36 cardiocranial syndrome 10.8 CRP CXCL8 ELANE
37 gastric ulcer 10.8 CTLA4 IFNG TNF
38 biemond syndrome 10.8 HLA-DPB1 TNF
39 cicatricial lagophthalmos 10.8 CRP CXCL8 TNF
40 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 10.8 CCR5 IFNG TNF
41 conduct disorder 10.8 IFNG SELE TNF
42 autoimmune polyglandular syndrome type 3 10.8 IFNG TNF
43 left bundle branch hemiblock 10.8 HLA-DPB1 IFNG TNF
44 anencephaly and spina bifida x-linked 10.8 CTLA4 MPO SERPINA1 TNF
45 atypical lichen myxedematosus 10.8 CCR5 PTPN22 TNF
46 myeloid sarcoma 10.8 CRP CXCL8 TNF
47 prostate calculus 10.8 PRTN3 TNF
48 mycobacterium kansasii 10.8 CXCL8 SERPINA1 TNF
49 plantar wart 10.8 CXCL8 IFNG TNF
50 lymphoma 10.8 CTLA4 IFNG PRTN3 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 visual impairment 56 32 occasional (7.5%) Occasional (29-5%) HP:0000505
2 recurrent respiratory infections 56 32 hallmark (90%) Very frequent (99-80%) HP:0002205
3 headache 56 32 occasional (7.5%) Occasional (29-5%) HP:0002315
4 seizures 56 32 occasional (7.5%) Occasional (29-5%) HP:0001250
5 hematuria 56 32 hallmark (90%) Very frequent (99-80%) HP:0000790
6 proteinuria 56 32 frequent (33%) Frequent (79-30%) HP:0000093
7 proptosis 56 32 occasional (7.5%) Occasional (29-5%) HP:0000520
8 fatigue 56 32 hallmark (90%) Very frequent (99-80%) HP:0012378
9 hydronephrosis 56 32 occasional (7.5%) Occasional (29-5%) HP:0000126
10 renal insufficiency 56 32 occasional (7.5%) Occasional (29-5%) HP:0000083
11 pancreatitis 56 32 occasional (7.5%) Occasional (29-5%) HP:0001733
12 pericarditis 56 32 occasional (7.5%) Occasional (29-5%) HP:0001701
13 pleuritis 56 32 occasional (7.5%) Occasional (29-5%) HP:0002102
14 hypertension 56 32 occasional (7.5%) Occasional (29-5%) HP:0000822
15 myalgia 56 32 occasional (7.5%) Occasional (29-5%) HP:0003326
16 epistaxis 56 32 hallmark (90%) Very frequent (99-80%) HP:0000421
17 purpura 56 32 occasional (7.5%) Occasional (29-5%) HP:0000979
18 arrhythmia 56 32 occasional (7.5%) Occasional (29-5%) HP:0011675
19 hemiplegia 56 32 occasional (7.5%) Occasional (29-5%) HP:0002301
20 fever 56 32 hallmark (90%) Very frequent (99-80%) HP:0001945
21 respiratory insufficiency 56 32 frequent (33%) Frequent (79-30%) HP:0002093
22 abdominal pain 56 32 frequent (33%) Frequent (79-30%) HP:0002027
23 venous thrombosis 56 32 occasional (7.5%) Occasional (29-5%) HP:0004936
24 cough 56 32 frequent (33%) Frequent (79-30%) HP:0012735
25 intestinal obstruction 56 32 occasional (7.5%) Occasional (29-5%) HP:0005214
26 diabetes insipidus 56 32 occasional (7.5%) Occasional (29-5%) HP:0000873
27 sensory neuropathy 56 32 occasional (7.5%) Occasional (29-5%) HP:0000763
28 weight loss 56 32 hallmark (90%) Very frequent (99-80%) HP:0001824
29 vasculitis 56 32 hallmark (90%) Very frequent (99-80%) HP:0002633
30 sensorineural hearing impairment 56 32 occasional (7.5%) Occasional (29-5%) HP:0000407
31 hemoptysis 56 32 frequent (33%) Frequent (79-30%) HP:0002105
32 retinopathy 56 32 occasional (7.5%) Occasional (29-5%) HP:0000488
33 gangrene 56 32 occasional (7.5%) Occasional (29-5%) HP:0100758
34 periorbital edema 56 32 frequent (33%) Frequent (79-30%) HP:0100539
35 skin rash 56 32 frequent (33%) Frequent (79-30%) HP:0000988
36 chest pain 56 32 frequent (33%) Frequent (79-30%) HP:0100749
37 arthralgia 56 32 hallmark (90%) Very frequent (99-80%) HP:0002829
38 pulmonary fibrosis 56 32 frequent (33%) Frequent (79-30%) HP:0002206
39 pulmonary infiltrates 56 32 hallmark (90%) Very frequent (99-80%) HP:0002113
40 chronic obstructive pulmonary disease 56 32 frequent (33%) Frequent (79-30%) HP:0006510
41 chronic otitis media 56 32 occasional (7.5%) Occasional (29-5%) HP:0000389
42 cranial nerve paralysis 56 32 occasional (7.5%) Occasional (29-5%) HP:0006824
43 sinusitis 56 32 hallmark (90%) Very frequent (99-80%) HP:0000246
44 cerebral ischemia 56 32 hallmark (90%) Very frequent (99-80%) HP:0002637
45 meningitis 56 32 occasional (7.5%) Occasional (29-5%) HP:0001287
46 angina pectoris 56 32 occasional (7.5%) Occasional (29-5%) HP:0001681
47 gastrointestinal hemorrhage 56 32 occasional (7.5%) Occasional (29-5%) HP:0002239
48 elevated erythrocyte sedimentation rate 56 32 frequent (33%) Frequent (79-30%) HP:0003565
49 ureteral stenosis 56 32 occasional (7.5%) Occasional (29-5%) HP:0000071
50 prostatitis 56 32 occasional (7.5%) Occasional (29-5%) HP:0000024

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 TNF CCR5 CD177 CD80 CTLA4 CTSG
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 103)
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 58-05-9 143 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-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1
25 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3
26 Autonomic Agents Phase 4,Phase 2,Phase 3
27 Dermatologic Agents Phase 4,Phase 3,Phase 2
28 Folic Acid Antagonists Phase 4,Phase 3,Phase 2
29 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1
30 glucocorticoids Phase 4,Phase 2,Phase 3
31 Hormone Antagonists Phase 4,Phase 2,Phase 3
32 Hormones Phase 4,Phase 2,Phase 3,Phase 1
33 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3
34 Methylprednisolone acetate Phase 4,Phase 2,Phase 3
35 Methylprednisolone Hemisuccinate Phase 4,Phase 2,Phase 3
36 Neuroprotective Agents Phase 4,Phase 2,Phase 3
37 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2
38 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1
39 Prednisolone acetate Phase 4,Phase 2,Phase 3
40 Prednisolone hemisuccinate Phase 4,Phase 2,Phase 3
41 Prednisolone phosphate Phase 4,Phase 2,Phase 3
42 Protective Agents Phase 4,Phase 2,Phase 3
43 Vitamin B Complex Phase 4,Phase 3,Phase 2
44 Tin Fluorides Phase 4
45 Folate Nutraceutical Phase 4,Phase 3,Phase 2
46 Vitamin B9 Nutraceutical Phase 4,Phase 3,Phase 2
47 Omega 3 Fatty Acid Nutraceutical Phase 4
48
Etanercept Approved, Investigational Phase 2, Phase 3,Phase 1 185243-69-0
49
Gemcitabine Approved Phase 3,Phase 1,Phase 2 95058-81-4 60750
50
belimumab Approved Phase 3 356547-88-1 5957 10451420

Interventional clinical trials:

(show top 50) (show all 115)

id 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 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 Methylprednisolone;Prednisone;Rituximab
5 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Recruiting NCT02626845 Phase 4 Rituximab
6 Low-dose Glucocorticoid Vasculitis Induction Study Recruiting NCT02198248 Phase 4 Rituximab;Glucocorticoids
7 Pilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis Active, not recruiting NCT02169219 Phase 4 Glucocorticoids;Rituximab
8 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
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 Prevention of Glucocorticoid-Induced Osteoporosis in Rheumatic Diseases: Alendronate Versus Alfacalcidol. Completed NCT00138983 Phase 3 Alendronate versus alfacalcidol (1-alpha OH vitamin D)
23 Rituximab in Eosinophilic Granulomatosis With Polyangiitis Recruiting NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
24 Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) Recruiting NCT02108860 Phase 3 Abatacept;placebo
25 The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach Recruiting NCT01933724 Phase 3 5 mg prednisone;0 mg prednisone
26 The Assessment of Prednisone In Remission Trial - Centers of Excellence Approach Recruiting NCT01940094 Phase 3 Prednisone 5 mg/day;Prednisone 0 mg/day
27 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
28 Long-term Access Program (LAP) of Mepolizumab for Subjects Who Participated in Study MEA115921 Active, not recruiting NCT03298061 Phase 3 Mepolizumab
29 Plasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis Active, not recruiting NCT00987389 Phase 3 Glucocorticoids
30 Rituximab Vasculitis Maintenance Study Active, not recruiting NCT01697267 Phase 3 Azathioprine
31 Comparison Study of Two Rituximab Regimens in the Remission of ANCA Associated Vasculitis Active, not recruiting NCT01731561 Phase 3 Rituximab (Arm B);Rituximab (Arm A)
32 Comparison Between a Long Term and a Conventional Maintenance Treatment With Rituximab Active, not recruiting NCT02433522 Phase 3 rituximab;Placebo
33 Study of Sacituzumab Govitecan in Refractory/Relapsed Triple-Negative Breast Cancer Not yet recruiting NCT02574455 Phase 3 Sacituzumab govitecan;Eribulin;Capecitabine;Gemcitabine;Vinorelbine
34 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
35 Intravenous Immunoglobulin After Relapse in Vasculitis Terminated NCT00307658 Phase 3 Intravenous immunoglobulins (human immunoglobulins G)
36 Plasma Exchange for Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3 Intravenous methyl prednisolone;Methyl prednisolone
37 IMPROVE: Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis Terminated NCT00307645 Phase 3 Cyclophosphamide;Mycophenolate mofetil;Azathioprine;Prednisone (and methylprednisolone)
38 Phase 3 Trial of 90Y-Clivatuzumab Tetraxetan & Gemcitabine vs Placebo & Gemcitabine in Metastatic Pancreatic Cancer Terminated NCT01956812 Phase 3 IMMU-107;placebo;Gemcitabine
39 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
40 Improving Outcomes Following Limb Loss: PALS Plus Unknown status NCT00447655 Phase 1, Phase 2
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 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
44 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2 Gusperimus
45 Daclizumab to Treat Wegener's Granulomatosis Completed NCT00040248 Phase 2 Daclizumab
46 Abatacept in Treating Adults With Mild Relapsing Wegener's Granulomatosis Completed NCT00468208 Phase 1, Phase 2 Abatacept
47 Etanercept to Treat Wegener's Granulomatosis Completed NCT00001901 Phase 2 Etanercept
48 Treatment of Wegener's Granulomatosis With Cyclophosphamide Completed NCT00001155 Phase 2 cyclophosphamide
49 Comparison of Treatments to Maintain Disease Remission in Patients With Wegener's Granulomatosis and Related Vasculitis Syndromes Completed NCT00004567 Phase 2 Mycophenolate Mofetil
50 Steroids and Methotrexate to Treat Systemic Vasculitis Completed NCT00001256 Phase 2 prednisone and methotrexate

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

Publications for Wegener Granulomatosis

Articles related to Wegener Granulomatosis:

(show top 50) (show all 63)
id Title Authors Year
1
Imaging diagnosis of orbital Wegener granulomatosis: A rare case report. ( 28591026 )
2017
2
Sinonasal imaging findings in granulomatosis with polyangiitis (Wegener granulomatosis): A systematic review. ( 28234146 )
2017
3
Healing of leg ulcers associated with granulomatosis with polyangiitis (Wegener granulomatosis) after rituximab therapy. ( 28207017 )
2017
4
Granulomatosis with polyangiitis (Wegener granulomatosis): a proteinase-3 driven disease? ( 28495524 )
2017
5
Granulomatosis with Polyangiitis (Wegener Granulomatosis) with Unusual Presentation. ( 28260778 )
2017
6
Monoclonal B-cell Lymphocytosis in a Patient with Wegener Granulomatosis: A Case Report and Update on 2016 World Health Organization Classification. ( 27625103 )
2016
7
Malignant Pyoderma Associated with Granulomatosis with Polyangiitis (Wegener Granulomatosis) as a Unique Indication for Facial Vascularized Composite Allotransplantation: Part I. ( 27219252 )
2016
8
Sellar Wegener Granulomatosis Masquerading as Cabergoline-Resistant Prolactinoma. ( 27521731 )
2016
9
Image Gallery: Symmetrical necrosis of the breast as an inaugural manifestation of granulomatosis with polyangiitis (Wegener granulomatosis). ( 27790680 )
2016
10
Management Scheme for Cerebral Wegener Granulomatosis: An Unusual Pseudotumoral Skull Base Pathology. ( 27671887 )
2016
11
Orbital involvement as the initial presentation of Wegener granulomatosis in a 9-year-old girl: MR imaging findings. ( 27425807 )
2016
12
Tracheal Stenosis Because of Wegener Granulomatosis Misdiagnosed as Asthma. ( 27075424 )
2016
13
Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up. ( 28393729 )
2016
14
Nasal mucosa narrow band imaging in granulomatosis with polyangiitis (Wegener granulomatosis): A preliminary study. ( 25781561 )
2015
15
Airway Surgery in Tracheostomised Patients with Wegener Granulomatosis Leading to Subglottic Stenosis. ( 27366530 )
2015
16
Total nasal skeletal reconstruction disfigured by granulomatosis with polyangitis (wegener granulomatosis). ( 25750847 )
2015
17
Renal cell carcinoma metastatic to the orbit in a patient with Wegener granulomatosis. ( 25295684 )
2015
18
Wegener granulomatosis as possible cause of vertigo: case report and review. ( 26513951 )
2015
19
Wegener Granulomatosis: Otologic Manifestation as First Symptom. ( 26157503 )
2015
20
Wegener granulomatosis with supraglottic involvement. ( 26433716 )
2015
21
Wegener granulomatosis-associated optic perineuritis. ( 24144064 )
2014
22
Ultrasonographic imaging of the peripheral nerves in a Wegener granulomatosis patient with multiple mononeuropathies. ( 24811470 )
2014
23
Limited-Form Wegener Granulomatosis Case: Anaesthetic Approach and Literature Review. ( 27366454 )
2014
24
Changes in sinonasal mucosa in Wegener granulomatosis. ( 24616132 )
2014
25
Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings. ( 25076302 )
2014
26
Long-term Outcome of Airway Stenosis in Granulomatosis With Polyangiitis (Wegener Granulomatosis): An Observational Study. ( 25321076 )
2014
27
Coexistent pulmonary granulomatosis with polyangiitis (Wegener granulomatosis) and Crohn disease. ( 24525506 )
2014
28
An unusual orbital localization of wegener granulomatosis detected by 18F-FDG PET/CT. ( 23856831 )
2014
29
Granulomatosis with polyangiitis (Wegener granulomatosis) as a differential diagnosis of sternal osteomyelitis: the challenges in diagnosis. ( 24263148 )
2013
30
Pathology quiz case 3: Wegener granulomatosis. ( 23681045 )
2013
31
Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood. ( 23729544 )
2013
32
Clinical features and outcomes of 37 Argentinean patients with severe granulomatosis with polyangiitis (wegener granulomatosis). ( 23364664 )
2013
33
Neutrophil proteinase 3 and dipeptidyl peptidase I (cathepsin C) as pharmacological targets in granulomatosis with polyangiitis (Wegener granulomatosis). ( 23385856 )
2013
34
Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine. ( 24252079 )
2013
35
Wegener granulomatosis--rare case presentation. ( 23777030 )
2012
36
Palpable purpura, gingival ulcerations and joint pain in a 24-year-old man. Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis). ( 22726723 )
2012
37
Kidney involvement in a Wegener granulomatosis case. ( 23897571 )
2012
38
Wegener granulomatosis presenting as refractory otitis media: a case report. ( 22991991 )
2012
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
Granulomatous choroiditis in Wegener granulomatosis. ( 21482884 )
2011
42
Wegener granulomatosis (granulomatosis with polyangiitis): evolving concepts in treatment. ( 21674414 )
2011
43
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
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
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
46
Mucocutaneous leishmaniasis masquerading as Wegener granulomatosis. ( 20375822 )
2010
47
Isolated blue toe syndrome as the initial sign of Wegener granulomatosis. ( 20609674 )
2010
48
Wegener granulomatosis presenting as epididymitis. ( 19376569 )
2009
49
Labyrinthitis related to Wegener granulomatosis: magnetic resonance imaging findings. ( 18097336 )
2008
50
A hydrophobic patch on proteinase 3, the target of autoantibodies in Wegener granulomatosis, mediates membrane binding via NB1 receptors. ( 18854317 )
2008

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 25)
id 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 12.43 CTLA4 IFNG MPO PTPN22 TNF
5
Show member pathways
12.33 CCR5 CTLA4 IFNG TNF
6
Show member pathways
12.27 BPI CTSG ELANE PRTN3
7
Show member pathways
12.16 CCR5 HLA-DPB1 IFNG TNF
8 12.13 CXCL8 ELANE MPO RXRB
9 12.01 CD80 CTLA4 HLA-DPB1 SELE
10 11.85 CD80 IFNG MPO TNF
11 11.74 CTSG CXCL8 IFNG TNF
12 11.7 CXCL8 IFNG SELE
13 11.67 ELANE MPO PRTN3
14 11.62 CD80 CTLA4 PTPN22
15
Show member pathways
11.59 CTLA4 CXCL8 IFNG TNF
16 11.48 CXCL8 IFNG SELE
17 11.47 CXCL8 ELN SERPINA1 TNF
18 11.42 ELANE MPO TNF
19
Show member pathways
11.42 CXCL8 IFNG SELE TNF
20 11.29 ELANE ELN SELE
21 11.25 CCR5 CD80 CXCL8 TNF
22 11.23 IFNG SELE TNF
23 11.15 CXCL8 IFNG SELE TNF
24 11.05 CD80 CTLA4 CXCL8 HLA-DPB1 IFNG TNF
25 10.89 CRP CXCL8 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 9.9 BPI CRP CTSG CXCL8 ELANE ELN
2 cell surface GO:0009986 9.85 CCR5 CD80 CTSG ELANE HLA-DPB1 TNF
3 extracellular space GO:0005615 9.7 BPI CRP CTSG CXCL8 ELANE IFNG
4 external side of plasma membrane GO:0009897 9.65 CCR5 CD80 CTLA4 IFNG TNF
5 secretory granule GO:0030141 9.58 CTSG ELANE MPO
6 protein complex involved in cell adhesion GO:0098636 9.32 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 23)
id Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.92 CCR5 CRP CXCL8 SELE TNF
2 leukocyte migration GO:0050900 9.87 CD177 ELANE SELE TNF
3 neutrophil degranulation GO:0043312 9.87 BPI CD177 CTSG ELANE MPO PRTN3
4 response to lipopolysaccharide GO:0032496 9.8 CTSG ELANE MPO PTPN22 SELE
5 defense response to Gram-positive bacterium GO:0050830 9.79 CRP CTSG TNF
6 extracellular matrix disassembly GO:0022617 9.78 CTSG ELANE ELN
7 T cell costimulation GO:0031295 9.77 CD80 CTLA4 HLA-DPB1
8 immune response GO:0006955 9.76 BPI CCR5 CTLA4 CTSG CXCL8 HLA-DPB1
9 defense response to bacterium GO:0042742 9.73 BPI CTSG ELANE IFNG MPO TNF
10 positive regulation of T cell proliferation GO:0042102 9.72 CD80 HLA-DPB1 IFNG
11 calcium-mediated signaling GO:0019722 9.67 CCR5 CXCL8 SELE
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 leukocyte migration involved in inflammatory response GO:0002523 9.59 ELANE SELE
15 positive regulation of chemokine biosynthetic process GO:0045080 9.58 IFNG TNF
16 positive regulation of immune response GO:0050778 9.58 CTSG ELANE
17 negative regulation of lipid storage GO:0010888 9.56 CRP TNF
18 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.54 ELANE TNF
19 response to yeast GO:0001878 9.51 ELANE MPO
20 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.48 IFNG TNF
21 positive regulation of vitamin D biosynthetic process GO:0060557 9.43 IFNG TNF
22 cellular response to lipopolysaccharide GO:0071222 9.43 CCR5 CD80 CTSG CXCL8 IFNG TNF
23 negative regulation of growth of symbiont in host GO:0044130 9.02 CTSG ELANE IFNG MPO 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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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