WG
MCID: GRN037
MIFTS: 69

Granulomatosis with Polyangiitis (WG)

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

Aliases & Classifications for Granulomatosis with Polyangiitis

MalaCards integrated aliases for Granulomatosis with Polyangiitis:

Name: Granulomatosis with Polyangiitis 58 12 54 26 60 38 44 45 15 17
Gpa 58 54 26 60
Wegener Granulomatosis, Formerly 58 12
Wegener Granulomatosis 54 13
Wegener Granulomatosis, Formerly; Wg, Formerly 58
Necrotizing Respiratory Granulomatosis 12
Wegeners Granulomatosis 56
Midline Granulomatosis 54
Wg, Formerly 58
Wg 54

Characteristics:

Orphanet epidemiological data:

60
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 Granulomatosis with Polyangiitis

OMIM : 58 Granulomatosis with polyangiitis, formerly termed Wegener granulomatosis, 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), which 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 GPA 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)

MalaCards based summary : Granulomatosis with Polyangiitis, also known as gpa, is related to eosinophilic granulomatosis with polyangiitis and churg-strauss syndrome. An important gene associated with Granulomatosis with Polyangiitis is PRTN3 (Proteinase 3), and among its related pathways/superpathways are Innate Immune System and NF-kappaB Signaling. The drugs alemtuzumab and rituximab have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and kidney, and related phenotypes are recurrent respiratory infections and fever

Disease Ontology : 12 An autoimmune hypersensitivity disease that 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, and is \nlocated 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 : 26 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 formerly 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 : 54 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. 

MedlinePlus : 44 Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. The inflammation limits the flow of blood to important organs, causing damage. It can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs, and kidneys. The cause of GPA is unknown. It can affect people at any age. Men and women are equally affected. It is more common in whites. Symptoms may include joint pain, weakness, tiredness, and cold symptoms such as a runny nose that doesn't get better. Doctors use blood tests, chest X-rays, and biopsies to diagnose GPA and rule out other causes of the symptoms. Early treatment is important. Most people improve with medicines to slow or stop the inflammation. NIH: National Institute of Allergy and Infectious Diseases

Wikipedia : 77 Granulomatosis with polyangiitis (GPA), formerly known as Wegener''s Granulomatosis (WG), is a long-term... more...

Related Diseases for Granulomatosis with Polyangiitis

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

(show top 50) (show all 265)
# Related Disease Score Top Affiliating Genes
1 eosinophilic granulomatosis with polyangiitis 35.0 FCGR3B MPO
2 churg-strauss syndrome 33.1 HMGB1 MPO PRTN3
3 anca-associated vasculitis 32.8 CTLA4 HMGB1 MPO PRTN3 SERPINA1
4 rapidly progressive glomerulonephritis 32.7 ELANE MPO PRTN3
5 vasculitis 31.5 BPI CTSG ELANE MPO PRTN3 SERPINA1
6 glomerulonephritis 30.9 MPO PRTN3 TLR9
7 bone inflammation disease 30.4 PRTN3 PTPN22 TLR9
8 pneumonia 30.3 ELANE MPO TLR9
9 rheumatoid arthritis 30.1 CTLA4 HLA-DPB1 HMGB1 PRTN3 PTPN22
10 alpha-1-antitrypsin deficiency 29.7 BPI ELANE SERPINA1
11 ulcerative colitis 29.5 BPI MPO PRTN3
12 crescentic glomerulonephritis 29.4 BPI CTSG MPO
13 lung disease 29.3 CTSG ELANE MPO PRTN3 SERPINA1 TLR9
14 systemic lupus erythematosus 28.3 CTLA4 CTSG ELANE ETS1 FCGR3B HLA-DPA1
15 allergic angiitis 11.7
16 scleritis 11.6
17 retinal vasculitis 11.6
18 granulomatous mastitis 11.6
19 autoimmune inner ear disease 11.3
20 microscopic polyangiitis 10.6
21 asthma 10.4
22 myocarditis 10.4
23 neuropathy 10.4
24 monoclonal paraproteinemia 10.4 MPO PRTN3
25 pyoderma 10.3
26 thrombosis 10.3
27 igg4-related disease 10.3
28 pyoderma gangrenosum 10.3
29 diffuse alveolar hemorrhage 10.3
30 lymphoma 10.3
31 endocarditis 10.3
32 beryllium disease 10.3 HLA-DPA1 HLA-DPB1
33 lung cancer 10.2
34 aspergillosis 10.2
35 gingivitis 10.2
36 ischemia 10.2
37 lupus erythematosus 10.2
38 hypereosinophilic syndrome 10.2
39 takayasu arteritis 10.2
40 cerebral aneurysms 10.2 ELANE MPO
41 berylliosis 10.2 HLA-DPA1 HLA-DPB1
42 atrial standstill 1 10.2
43 arthritis 10.2
44 allergic bronchopulmonary aspergillosis 10.2
45 cholecystitis 10.2
46 rere-related disorders 10.2
47 chronic beryllium disease 10.2 HLA-DPA1 HLA-DPB1
48 bronchitis 10.2 ELANE MPO SERPINA1
49 myocardial infarction 10.1
50 crohn's disease 10.1

Graphical network of the top 20 diseases related to Granulomatosis with Polyangiitis:



Diseases related to Granulomatosis with Polyangiitis

Symptoms & Phenotypes for Granulomatosis with Polyangiitis

Human phenotypes related to Granulomatosis with Polyangiitis:

60 33 (show top 50) (show all 67)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 recurrent respiratory infections 60 33 hallmark (90%) Very frequent (99-80%) HP:0002205
2 fever 60 33 hallmark (90%) Very frequent (99-80%) HP:0001945
3 fatigue 60 33 hallmark (90%) Very frequent (99-80%) HP:0012378
4 arthralgia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002829
5 weight loss 60 33 hallmark (90%) Very frequent (99-80%) HP:0001824
6 sinusitis 60 33 hallmark (90%) Very frequent (99-80%) HP:0000246
7 autoimmunity 60 33 hallmark (90%) Very frequent (99-80%) HP:0002960
8 cerebral ischemia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002637
9 glomerulopathy 60 33 hallmark (90%) Very frequent (99-80%) HP:0100820
10 hematuria 60 33 hallmark (90%) Very frequent (99-80%) HP:0000790
11 epistaxis 60 33 hallmark (90%) Very frequent (99-80%) HP:0000421
12 vasculitis 60 33 hallmark (90%) Very frequent (99-80%) HP:0002633
13 pulmonary infiltrates 60 33 hallmark (90%) Very frequent (99-80%) HP:0002113
14 granulomatosis 60 33 hallmark (90%) Very frequent (99-80%) HP:0002955
15 abnormal oral cavity morphology 33 hallmark (90%) HP:0000163
16 nausea and vomiting 60 33 frequent (33%) Frequent (79-30%) HP:0002017
17 respiratory insufficiency 60 33 frequent (33%) Frequent (79-30%) HP:0002093
18 proteinuria 60 33 frequent (33%) Frequent (79-30%) HP:0000093
19 pulmonary fibrosis 60 33 frequent (33%) Frequent (79-30%) HP:0002206
20 abdominal pain 60 33 frequent (33%) Frequent (79-30%) HP:0002027
21 chronic obstructive pulmonary disease 60 33 frequent (33%) Frequent (79-30%) HP:0006510
22 hemoptysis 60 33 frequent (33%) Frequent (79-30%) HP:0002105
23 chest pain 60 33 frequent (33%) Frequent (79-30%) HP:0100749
24 skin rash 60 33 frequent (33%) Frequent (79-30%) HP:0000988
25 cough 60 33 frequent (33%) Frequent (79-30%) HP:0012735
26 recurrent intrapulmonary hemorrhage 60 33 frequent (33%) Frequent (79-30%) HP:0006535
27 periorbital edema 60 33 frequent (33%) Frequent (79-30%) HP:0100539
28 inflammatory abnormality of the eye 60 33 frequent (33%) Frequent (79-30%) HP:0100533
29 papule 60 33 frequent (33%) Frequent (79-30%) HP:0200034
30 elevated c-reactive protein level 60 33 frequent (33%) Frequent (79-30%) HP:0011227
31 abnormality of the hypothalamus-pituitary axis 60 33 frequent (33%) Frequent (79-30%) HP:0000864
32 elevated erythrocyte sedimentation rate 60 33 frequent (33%) Frequent (79-30%) HP:0003565
33 hypertension 60 33 occasional (7.5%) Occasional (29-5%) HP:0000822
34 seizures 60 33 occasional (7.5%) Occasional (29-5%) HP:0001250
35 chronic otitis media 60 33 occasional (7.5%) Occasional (29-5%) HP:0000389
36 sensorineural hearing impairment 60 33 occasional (7.5%) Occasional (29-5%) HP:0000407
37 visual impairment 60 33 occasional (7.5%) Occasional (29-5%) HP:0000505
38 renal insufficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0000083
39 retinopathy 60 33 occasional (7.5%) Occasional (29-5%) HP:0000488
40 cranial nerve paralysis 60 33 occasional (7.5%) Occasional (29-5%) HP:0006824
41 arrhythmia 60 33 occasional (7.5%) Occasional (29-5%) HP:0011675
42 angina pectoris 60 33 occasional (7.5%) Occasional (29-5%) HP:0001681
43 sensory neuropathy 60 33 occasional (7.5%) Occasional (29-5%) HP:0000763
44 venous thrombosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0004936
45 skin ulcer 60 33 occasional (7.5%) Occasional (29-5%) HP:0200042
46 pancreatitis 60 33 occasional (7.5%) Occasional (29-5%) HP:0001733
47 myalgia 60 33 occasional (7.5%) Occasional (29-5%) HP:0003326
48 intestinal obstruction 60 33 occasional (7.5%) Occasional (29-5%) HP:0005214
49 diabetes insipidus 60 33 occasional (7.5%) Occasional (29-5%) HP:0000873
50 purpura 60 33 occasional (7.5%) Occasional (29-5%) HP:0000979

Clinical features from OMIM:

608710

MGI Mouse Phenotypes related to Granulomatosis with Polyangiitis:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 CCR6 CTLA4 CTSG ELANE ETS1 FCGR3B
2 immune system MP:0005387 9.36 CCR6 CTLA4 CTSG ELANE ETS1 FCGR3B

Drugs & Therapeutics for Granulomatosis with Polyangiitis

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

(show top 50) (show all 131)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
alemtuzumab Approved, Investigational Phase 4,Phase 1 216503-57-0
2
rituximab Approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 174722-31-7 10201696
3
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 4,Phase 2,Phase 3,Not Applicable 2921-57-5
5
Prednisolone phosphate Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 302-25-0
6
Prednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 50-24-8 5755
7
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 53-03-2 5865
8
Methotrexate Approved Phase 4,Phase 3,Phase 2,Phase 1 1959-05-2, 59-05-2 126941
9
Mycophenolic acid Approved Phase 4,Phase 3,Phase 2,Phase 1 24280-93-1 446541
10
Cyclophosphamide Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 6055-19-2, 50-18-0 2907
11
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
12
leucovorin Approved Phase 4,Phase 3,Phase 2 58-05-9 6006 143
13
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2 59-30-3 6037
14
Prednisolone hemisuccinate Experimental Phase 4,Phase 2,Phase 3,Not Applicable 2920-86-7
15 Antineoplastic Agents, Immunological Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
16 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
17 Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
18 Immunologic Factors Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
19 Methylprednisolone Acetate Phase 4,Phase 2,Phase 3,Not Applicable
20 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
21 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
23 Hormones Phase 4,Phase 2,Phase 3,Not Applicable
24 Prednisolone acetate Phase 4,Phase 2,Phase 3,Not Applicable
25 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
26 Antineoplastic Agents, Alkylating Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
27 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1
28 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
29 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
30 Autonomic Agents Phase 4,Phase 2,Phase 3,Not Applicable
31 Vitamin B Complex Phase 4,Phase 3,Phase 2
32 Antimetabolites Phase 4,Phase 3,Phase 2,Phase 1
33 Immunosuppressive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
34 Protective Agents Phase 4,Phase 2,Phase 3,Not Applicable
35 Folic Acid Antagonists Phase 4,Phase 3,Phase 2
36 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
37 Neuroprotective Agents Phase 4,Phase 2,Phase 3,Not Applicable
38 Dermatologic Agents Phase 4,Phase 3,Phase 2,Not Applicable
39 Folate Phase 4,Phase 3,Phase 2
40 Vitamin B9 Phase 4,Phase 3,Phase 2
41 Antiemetics Phase 4,Phase 2,Phase 3,Not Applicable
42 Antitubercular Agents Phase 4,Phase 3,Phase 2,Phase 1
43 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2,Phase 1
44 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2
45 Alkylating Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
46 Antibiotics, Antitubercular Phase 4,Phase 3,Phase 2,Phase 1
47 Antibodies Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
48 Immunoglobulins Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
49 Antibodies, Antineutrophil Cytoplasmic Phase 4,Phase 3,Phase 2,Not Applicable
50 Tin Fluorides Phase 4

Interventional clinical trials:

(show top 50) (show all 129)
# Name Status NCT ID Phase Drugs
1 Alemtuzumab for ANCA Associated Refractory Vasculitis Unknown status NCT01405807 Phase 4 Alemtuzumab
2 Pilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis Completed NCT02169219 Phase 4 Glucocorticoids;Rituximab
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 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
5 A Study Evaluating the Safety and Efficacy of Rituximab in Combination With Glucocorticoids in Participants With Wegener's Granulomatosis or Microscopic Polyangitis Recruiting NCT02115997 Phase 4 Methylprednisolone;Prednisone;Rituximab
6 Low-dose Glucocorticoid Vasculitis Induction Study Recruiting NCT02198248 Phase 4 Rituximab;Glucocorticoids
7 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Terminated NCT02626845 Phase 4 Rituximab
8 Interventional Cryotherapy for the Eradication of Benign Airway Disease ("ICE the BAD") Terminated NCT00747461 Phase 4
9 Trial to Study the Effects of Supplementary Omega-3 on Serum C-Reactive Protein Levels Terminated NCT00578578 Phase 4
10 Prevention of Relapses in Proteinase 3 (PR3)-Anti-neutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis Terminated NCT00128895 Phase 4 azathioprine
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 A Study to Investigate Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis Completed NCT02020889 Phase 3 Placebo
17 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
18 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
19 Belimumab in Remission of VASculitis Completed NCT01663623 Phase 3 Azathioprine
20 Plasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis Completed NCT00987389 Phase 3 Glucocorticoids
21 Comparison Study of Two Rituximab Regimens in the Remission of ANCA Associated Vasculitis Completed NCT01731561 Phase 3 Rituximab (Arm B);Rituximab (Arm A)
22 Efficacy Study of Two Treatments in the Remission of Vasculitis Completed NCT00748644 Phase 3 Rituximab;Azathioprine
23 Pulse Versus Continuous Cyclophosphamide for Induction of Remission in ANCA-Associated Vasculitides Completed NCT00430105 Phase 2, Phase 3 cyclophosphamide
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 Abatacept in earLy Onset Polymyalgia Rheumatica: Study ALORS Active, not recruiting NCT03632187 Phase 3 Abatacept;Placebos
35 Comparison Between a Long Term and a Conventional Maintenance Treatment With Rituximab Active, not recruiting NCT02433522 Phase 3 rituximab;Placebo
36 Cyclophosphamide vs. Infliximab for Refractory Idiopathic Scleritis (CIRIS) Not yet recruiting NCT03088293 Phase 3 Infliximab;Cyclophosphamide
37 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
38 Intravenous Immunoglobulin After Relapse in Vasculitis Terminated NCT00307658 Phase 3 Intravenous immunoglobulins (human immunoglobulins G)
39 Plasma Exchange for Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3 Intravenous methyl prednisolone;Methyl prednisolone
40 IMPROVE: Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis Terminated NCT00307645 Phase 3 Cyclophosphamide;Mycophenolate mofetil;Azathioprine;Prednisone (and methylprednisolone)
41 Phase 3 Trial of 90Y-Clivatuzumab Tetraxetan & Gemcitabine vs Placebo & Gemcitabine in Metastatic Pancreatic Cancer Terminated NCT01956812 Phase 3 IMMU-107;placebo;Gemcitabine
42 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
43 Phase Ib Study of SC Milatuzumab in SLE Unknown status NCT01845740 Phase 1, Phase 2 milatuzumab;Placebo
44 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
45 Study of Veltuzumab (hA20) at Different Doses in Patients With ITP Unknown status NCT00547066 Phase 1, Phase 2
46 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
47 A Phase IIa Study of Intravenous Rituximab in Pediatric Participants With Severe Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis Completed NCT01750697 Phase 2 Rituximab
48 Abatacept in Treating Adults With Mild Relapsing Wegener's Granulomatosis Completed NCT00468208 Phase 1, Phase 2 Abatacept
49 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2 Gusperimus
50 Daclizumab to Treat Wegener's Granulomatosis Completed NCT00040248 Phase 2 Daclizumab

Search NIH Clinical Center for Granulomatosis with Polyangiitis

Cochrane evidence based reviews: granulomatosis with polyangiitis

Genetic Tests for Granulomatosis with Polyangiitis

Anatomical Context for Granulomatosis with Polyangiitis

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

20
Lung, Skin, Kidney

MalaCards organs/tissues related to Granulomatosis with Polyangiitis:

42
Lung, Kidney, Skin, Neutrophil, Testes, Trachea, Heart

Publications for Granulomatosis with Polyangiitis

Articles related to Granulomatosis with Polyangiitis:

(show top 50) (show all 1421)
# Title Authors Year
1
Chronic pulmonary aspergillosis may cause eosinophilic granulomatosis with polyangiitis via allergic bronchopulmonary aspergillosis. ( 30800324 )
2019
2
Limited GPA and Alpha-1 Antitrypsin Deficiency in a Pediatric Patient. ( 30824654 )
2019
3
Aortitis and pachymeningitis: an unusual combination in granulomatosis with polyangiitis (myeloperoxidase-associated vasculitis). ( 30696638 )
2019
4
Serum cytokine and chemokine levels in patients with eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, or eosinophilic asthma. ( 30652675 )
2019
5
Refractory diffuse alveolar hemorrhage caused by eosinophilic granulomatosis with polyangiitis in the absence of elevated biomarkers treated successfully by rituximab and mepolizumab: A case report. ( 30603598 )
2019
6
IgG4-Related Disease and Eosinophilic Granulomatosis with Polyangiitis: Similarity or Coexistence? ( 30772965 )
2019
7
Childhood-onset Eosinophilic Granulomatosis with Polyangiitis with a Vulvar Granuloma: A Case Report and Review of the Literature. ( 30904627 )
2019
8
Anti-neutrophil cytoplasmic antibody-positive eosinophilic granulomatosis with polyangiitis: can it cause membranous nephropathy? ( 30907692 )
2019
9
Flare of eosinophilic granulomatosis with polyangiitis related to pregnancy: Case report and review of the literature. ( 30456168 )
2019
10
Thrombotic microangiopathy in a patient with eosinophilic granulomatosis with polyangiitis: case-based review. ( 30554307 )
2019
11
Pregnancy in a patient with eosinophilic granulomatosis with polyangiitis. ( 30634882 )
2019
12
A Perianal Mass Associated With Eosinophilic Granulomatosis With Polyangiitis: Challenge. ( 30640763 )
2019
13
PET/CT Findings of Granulomatosis With Polyangiitis Presenting as a Solitary Pulmonary Nodule and Mimicking Lung Cancer. ( 30664544 )
2019
14
IgG4:IgG RNA ratio differentiates active disease from remission in granulomatosis with polyangiitis: a new disease activity marker? A cross-sectional and longitudinal study. ( 30704507 )
2019
15
Clinical significance of ocular manifestations in granulomatosis with polyangiitis: association with sinonasal involvement and damage. ( 30706192 )
2019
16
Isolated choroid plexus involvement in a case of granulomatosis with polyangiitis negative for antineutrophil cytoplasmic antibodies (ANCA). ( 30708210 )
2019
17
Development of CT-based methods for longitudinal analyses of paranasal sinus osteitis in granulomatosis with polyangiitis. ( 30717680 )
2019
18
Incidence and prevalence of granulomatosis with polyangiitis and microscopic polyangiitis in health management organization in Argentina: a 15-year study. ( 30746579 )
2019
19
Granulomatosis With Polyangiitis Induced by Infection. ( 30775632 )
2019
20
Anemia Combined Significantly Increased High-Sensitivity C Reactive Protein and Lung Lesions Lead to the Diagnosis of Granulomatosis with Polyangiitis Proven by Lung Biopsy and Anti-neutrophil Cytoplasmic Antibody Test. ( 30775877 )
2019
21
Rituximab as an effective and probably safe treatment for granulomatosis with polyangiitis (Wegener's Granulomatosis). ( 30785092 )
2019
22
Rare presentation of granulomatosis with polyangiitis. ( 30798274 )
2019
23
Echocardiogram: The GPS to GPA's Heart (Granulomatosis with Polyangiitis). ( 30800485 )
2019
24
Transthoracic Ultrasound-Guided Fine Needle Aspiration Biopsy in the Differential Diagnosis of Granulomatosis With Polyangiitis. ( 30801333 )
2019
25
Hypophysis Involvement in Granulomatosis with Polyangiitis. ( 30816641 )
2019
26
'Carpal tunnel syndrome' and 'tennis elbow' as prodromes for granulomatosis with polyangiitis (formerly Wegener's granulomatosis). ( 30824462 )
2019
27
Comment on: A case of granulomatosis with polyangiitis complicated by two opportunistic infections. ( 30835088 )
2019
28
Ocular involvement in granulomatosis with polyangiitis: A single-center cohort study on 63 patients. ( 30844550 )
2019
29
Granulomatosis with polyangiitis in a patient with biopsy-proven IgG4-related pulmonary disease and coincident small cell lung cancer. ( 30872335 )
2019
30
Ocular Manifestations of Granulomatosis with Polyangiitis: A Review of the Literature. ( 30875067 )
2019
31
Should nasal biopsy inevitably be performed for classifying granulomatosis with polyangiitis in patients with rhinosinusitis? A retrospective chart review study. ( 30887162 )
2019
32
Subglottic Stenosis in Granulomatosis With Polyangiitis. ( 30904050 )
2019
33
All-Cause and Cause-Specific Mortality in Patients With Granulomatosis With Polyangiitis: A Population-Based Study. ( 29692001 )
2019
34
Breast involvement in granulomatosis with polyangiitis. ( 29800711 )
2019
35
Frequency, treatment, evolution, and factors associated with the presence of tracheobronchial stenoses in granulomatosis with polyangiitis. Retrospective analysis of a case series from a single respiratory referral center. ( 29891418 )
2019
36
Is granulomatosis with polyangiitis in Asia different from the West? ( 30338654 )
2019
37
Changes in the composition of the upper respiratory tract microbial community in granulomatosis with polyangiitis. ( 30420263 )
2019
38
Molecular targeted therapies for microscopic polyangiitis and granulomatosis with polyangiitis. ( 30613065 )
2019
39
Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis): Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome. ( 30633023 )
2019
40
Circulating CD24hiCD38hi regulatory B cells correlate inversely with the ThEM17 cell frequency in granulomatosis with polyangiitis patients. ( 30649475 )
2019
41
Relapsing granulomatosis with polyangiitis provoked by trauma: A case report. ( 30653170 )
2019
42
Are PR3 positive and MPO positive GPA the same disease? ( 29611345 )
2019
43
Hexagonal Layered Polymeric Nitrogen Phase Synthesized near 250 GPa. ( 30822079 )
2019
44
Evidence for Crystalline Structure in Dynamically-Compressed Polyethylene up to 200 GPa. ( 30862904 )
2019
45
Orbital granulomatosis with polyangiitis masquerading as invasive fungal sinusitis. ( 29303390 )
2018
46
Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRareAr cohort. ( 29943913 )
2018
47
Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis. ( 29436199 )
2018
48
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
49
Eosinophilic Granulomatosis With Polyangiitis: Newer Therapies. ( 29611001 )
2018
50
Multiple Cerebral Infarctions Due to Patent Foramen Ovale in a Patient with Eosinophilic Granulomatosis with Polyangiitis. ( 29373226 )
2018

Variations for Granulomatosis with Polyangiitis

Copy number variations for Granulomatosis with Polyangiitis 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 Granulomatosis with Polyangiitis

Search GEO for disease gene expression data for Granulomatosis with Polyangiitis.

Pathways for Granulomatosis with Polyangiitis

GO Terms for Granulomatosis with Polyangiitis

Cellular components related to Granulomatosis with Polyangiitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.91 BPI COL11A2 CTSG ELANE HMGB1 MPO
2 extracellular region GO:0005576 9.85 BPI COL11A2 CTSG ELANE FCGR3B HMGB1
3 cell surface GO:0009986 9.8 CCR6 CTSG ELANE HLA-DPA1 HLA-DPB1 HMGB1
4 secretory granule GO:0030141 9.54 CTSG ELANE MPO
5 MHC class II protein complex GO:0042613 9.4 HLA-DPA1 HLA-DPB1
6 collagen-containing extracellular matrix GO:0062023 9.35 COL11A2 CTSG ELANE PRTN3 SERPINA1
7 azurophil granule lumen GO:0035578 9.02 BPI CTSG ELANE MPO PRTN3

Biological processes related to Granulomatosis with Polyangiitis according to GeneCards Suite gene sharing:

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.8 CTLA4 ETS1 HLA-DPA1 HLA-DPB1 HMGB1 PTPN22
2 response to lipopolysaccharide GO:0032496 9.78 CTSG ELANE MPO PTPN22
3 T cell receptor signaling pathway GO:0050852 9.77 HLA-DPA1 HLA-DPB1 PTPN22
4 defense response to bacterium GO:0042742 9.77 BPI CTSG ELANE MPO TLR9
5 defense response to Gram-negative bacterium GO:0050829 9.74 BPI CTSG TLR9
6 dendritic cell chemotaxis GO:0002407 9.58 CCR6 HMGB1
7 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.58 HLA-DPA1 HLA-DPB1
8 defense response to fungus GO:0050832 9.58 CTSG ELANE MPO
9 positive regulation of immune response GO:0050778 9.56 CTSG ELANE
10 neutrophil degranulation GO:0043312 9.56 BPI CTSG ELANE FCGR3B HMGB1 MPO
11 positive regulation of interferon-alpha secretion GO:1902741 9.55 HMGB1 PTPN22
12 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.54 ELANE TLR9
13 leukocyte migration involved in inflammatory response GO:0002523 9.51 CCR6 ELANE
14 negative regulation of growth of symbiont in host GO:0044130 9.5 CTSG ELANE MPO
15 negative regulation of interleukin-8 production GO:0032717 9.49 BPI TLR9
16 response to yeast GO:0001878 9.46 ELANE MPO
17 antimicrobial humoral response GO:0019730 9.46 BPI CTSG ELANE PRTN3
18 positive regulation of toll-like receptor 9 signaling pathway GO:0034165 9.43 HMGB1 PTPN22
19 immune response GO:0006955 9.28 BPI CCR6 CTLA4 CTSG ETS1 FCGR3B
20 positive regulation of toll-like receptor 4 signaling pathway GO:0034145 9.22 PTPN22

Sources for Granulomatosis with Polyangiitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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