WG
MCID: GRN037
MIFTS: 68

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 57 12 53 25 59 37 43 44 15
Gpa 57 53 25 59
Wegener Granulomatosis, Formerly 57 12
Wegener Granulomatosis 53 13
Wegener Granulomatosis, Formerly; Wg, Formerly 57
Necrotizing Respiratory Granulomatosis 12
Wegeners Granulomatosis 55
Midline Granulomatosis 53
Wg, Formerly 57
Wg 53

Characteristics:

Orphanet epidemiological data:

59
granulomatosis with polyangiitis
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe),1-5/10000 (Sweden),1-9/1000000 (Germany),1-9/100000 (France),1-9/100000 (Sweden),1-9/100000 (Norway),1-9/100000 (Australia),1-9/100000 (New Zealand),1-9/100000 (United States),1-9/100000 (China),1-9/1000000 (Lithuania),1-9/1000000 (Greece),1-9/1000000 (Italy),1-9/1000000 (Canada),1-9/1000000 (Japan),<1/1000000 (Taiwan, Province of China),1-9/100000 (Italy),1-5/10000 (Denmark); Age of onset: All ages; Age of death: any age;

Classifications:



Summaries for Granulomatosis with Polyangiitis

OMIM : 57 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 hypertension and seizures

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

MedlinePlus : 43 Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. The inflammation limits the flow of blood to important organs, causing damage. It can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs, and kidneys. The cause of GPA is unknown. It can affect people at any age. Men and women are equally affected. It is more common in whites. Symptoms may include joint pain, weakness, tiredness, and cold symptoms such as a runny nose that doesn't get better. Doctors use blood tests, chest X-rays, and biopsies to diagnose GPA and rule out other causes of the symptoms. Early treatment is important. Most people improve with medicines to slow or stop the inflammation. NIH: National Institute of Allergy and Infectious Diseases

Wikipedia : 76 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 251)
# Related Disease Score Top Affiliating Genes
1 eosinophilic granulomatosis with polyangiitis 34.7 FCGR3B MPO
2 churg-strauss syndrome 32.8 HMGB1 MPO PRTN3
3 anca-associated vasculitis 32.7 CTLA4 HMGB1 MPO PRTN3 SERPINA1
4 rapidly progressive glomerulonephritis 32.5 ELANE MPO PRTN3
5 vasculitis 31.8 BPI CTSG ELANE MPO PRTN3 SERPINA1
6 microscopic polyangiitis 31.3 FCGR3B MPO
7 glomerulonephritis 30.6 MPO PRTN3 TLR9
8 pneumonia 30.4 ELANE MPO TLR9
9 rheumatoid arthritis 30.0 CTLA4 HLA-DPB1 HMGB1 PRTN3 PTPN22
10 bone inflammation disease 30.0 PRTN3 PTPN22 TLR9
11 alpha-1-antitrypsin deficiency 29.8 BPI ELANE SERPINA1
12 ulcerative colitis 29.6 BPI MPO PRTN3
13 crescentic glomerulonephritis 29.6 BPI CTSG MPO
14 lung disease 29.5 CTSG ELANE MPO PRTN3 SERPINA1 TLR9
15 systemic lupus erythematosus 29.1 CTLA4 CTSG ELANE ETS1 FCGR3B HLA-DPB1
16 inflammatory bowel disease 28.9 BPI CTLA4 CTSG ELANE MPO PRTN3
17 allergic angiitis 11.7
18 scleritis 11.6
19 retinal vasculitis 11.6
20 granulomatous mastitis 11.6
21 autoimmune inner ear disease 11.3
22 neuropathy 10.4
23 asthma 10.3
24 pyoderma 10.3
25 myocarditis 10.3
26 pyoderma gangrenosum 10.3
27 lymphoma 10.3
28 thrombosis 10.3
29 endocarditis 10.3
30 diffuse alveolar hemorrhage 10.3
31 monoclonal paraproteinemia 10.2 MPO PRTN3
32 temporal arteritis 10.2
33 gingivitis 10.2
34 ischemia 10.2
35 lupus erythematosus 10.2
36 igg4-related disease 10.2
37 aspergillosis 10.2
38 arthritis 10.2
39 cholecystitis 10.2
40 hypereosinophilic syndrome 10.2
41 cerebral aneurysms 10.1 ELANE MPO
42 immune system disease 10.1 CTLA4 PRTN3 TLR9
43 lung cancer 10.1
44 crohn's disease 10.1
45 hepatitis 10.1
46 colitis 10.1
47 osteomyelitis 10.1
48 allergic bronchopulmonary aspergillosis 10.1
49 polyneuropathy 10.1
50 facial paralysis 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

Clinical features from OMIM:

608710

Human phenotypes related to Granulomatosis with Polyangiitis:

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

MGI Mouse Phenotypes related to Granulomatosis with Polyangiitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.73 CTLA4 CTSG ELANE ETS1 FCGR3B IL15RA
2 immune system MP:0005387 9.4 CTLA4 CTSG ELANE ETS1 FCGR3B IL15RA

Drugs & Therapeutics for Granulomatosis with Polyangiitis

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

(show top 50) (show all 126)
# 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 3,Phase 2,Phase 1,Not Applicable 174722-31-7 10201696
3
Azathioprine Approved Phase 4,Phase 3,Phase 2 446-86-6 2265
4
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 83-43-2 6741
5
Prednisolone phosphate Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 302-25-0
6
Methylprednisolone hemisuccinate Approved Phase 4,Phase 2,Phase 3,Not Applicable 2921-57-5
7
Prednisone Approved, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 53-03-2 5865
8
Prednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 50-24-8 5755
9
Cyclophosphamide Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 6055-19-2, 50-18-0 2907
10
leucovorin Approved Phase 4,Phase 3,Phase 2 58-05-9 6006 143
11
Mycophenolic acid Approved Phase 4,Phase 3,Phase 2,Phase 1 24280-93-1 446541
12
Methotrexate Approved Phase 4,Phase 3,Phase 2,Phase 1 59-05-2, 1959-05-2 126941
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 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
16 Immunosuppressive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
17 Antimetabolites Phase 4,Phase 3,Phase 2,Phase 1
18 Antimetabolites, Antineoplastic Phase 4,Phase 3,Phase 2,Phase 1
19 glucocorticoids Phase 4,Phase 3,Phase 2,Not Applicable
20 Immunologic Factors Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
21 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Not Applicable
23 Hormone Antagonists Phase 4,Phase 3,Phase 2,Not Applicable
24 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
25 Autonomic Agents Phase 4,Phase 2,Phase 3,Not Applicable
26 Hormones Phase 4,Phase 3,Phase 2,Not Applicable
27 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
28 Prednisolone acetate Phase 4,Phase 2,Phase 3,Not Applicable
29 Methylprednisolone acetate Phase 4,Phase 2,Phase 3,Not Applicable
30 Antiemetics Phase 4,Phase 2,Phase 3,Not Applicable
31 Neuroprotective Agents Phase 4,Phase 2,Phase 3,Not Applicable
32 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
33 Protective Agents Phase 4,Phase 3,Phase 2,Not Applicable
34 Antineoplastic Agents, Alkylating Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
35 Vitamin B9 Phase 4,Phase 3,Phase 2
36 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
37 Alkylating Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
38 Antitubercular Agents Phase 4,Phase 3,Phase 2,Phase 1
39 Vitamin B Complex Phase 4,Phase 3,Phase 2
40 Antibiotics, Antitubercular Phase 4,Phase 3,Phase 2,Phase 1
41 Folic Acid Antagonists Phase 4,Phase 3,Phase 2
42 Dermatologic Agents Phase 4,Phase 3,Phase 2,Not Applicable
43 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1
44 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2
45 Folate Phase 4,Phase 3,Phase 2
46 Immunoglobulins Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
47 Antibodies, Antineutrophil Cytoplasmic Phase 4,Phase 3,Phase 2,Not Applicable
48 Antibodies Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
49 Tin Fluorides Phase 4
50 Omega 3 Fatty Acid Phase 4

Interventional clinical trials:

(show top 50) (show all 128)
# Name Status NCT ID Phase Drugs
1 Alemtuzumab for ANCA Associated Refractory Vasculitis Unknown status NCT01405807 Phase 4 Alemtuzumab
2 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
3 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Terminated NCT02626845 Phase 4 Rituximab
4 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
5 Pilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis Completed NCT02169219 Phase 4 Glucocorticoids;Rituximab
6 Low-dose Glucocorticoid Vasculitis Induction Study Recruiting NCT02198248 Phase 4 Rituximab;Glucocorticoids
7 Interventional Cryotherapy for the Eradication of Benign Airway Disease ("ICE the BAD") Terminated NCT00747461 Phase 4
8 Trial to Study the Effects of Supplementary Omega-3 on Serum C-Reactive Protein Levels Terminated NCT00578578 Phase 4
9 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Completed NCT00307671 Phase 4 prednisone, methylprednisolone,cyclophosphamides;Cyclophosphamide, Azathioprine,prednisone,methylprednisolone;Mycophenolate mofetil,methotrexate
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 Rituximab in Eosinophilic Granulomatosis With Polyangiitis Recruiting NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
16 Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) Recruiting NCT02108860 Phase 3 Abatacept;placebo
17 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
18 Etanercept for Wegener's Granulomatosis Completed NCT00005007 Phase 2, Phase 3 Etanercept
19 A Study to Investigate Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis Completed NCT02020889 Phase 3 Placebo
20 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
21 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
22 Long-term Access Program (LAP) of Mepolizumab for Subjects Who Participated in Study MEA115921 Active, not recruiting NCT03298061 Phase 3 Mepolizumab;Prednisolone
23 The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach Recruiting NCT01933724 Phase 3 5 mg prednisone;0 mg prednisone
24 The Assessment of Prednisone In Remission Trial - Centers of Excellence Approach Recruiting NCT01940094 Phase 3 5 mg Prednisone;0 mg Prednisone
25 Belimumab in Remission of VASculitis Completed NCT01663623 Phase 3 Azathioprine
26 Plasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis Completed NCT00987389 Phase 3 Glucocorticoids
27 Comparison Study of Two Rituximab Regimens in the Remission of ANCA Associated Vasculitis Completed NCT01731561 Phase 3 Rituximab (Arm B);Rituximab (Arm A)
28 Intravenous Immunoglobulin After Relapse in Vasculitis Terminated NCT00307658 Phase 3 Intravenous immunoglobulins (human immunoglobulins G)
29 Efficacy Study of Two Treatments in the Remission of Vasculitis Completed NCT00748644 Phase 3 Rituximab;Azathioprine
30 Plasma Exchange for Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3 Intravenous methyl prednisolone;Methyl prednisolone
31 Pulse Versus Continuous Cyclophosphamide for Induction of Remission in ANCA-Associated Vasculitides Completed NCT00430105 Phase 2, Phase 3 cyclophosphamide
32 Rituximab Vasculitis Maintenance Study Active, not recruiting NCT01697267 Phase 3 Azathioprine
33 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
34 Cyclophosphamide vs. Infliximab for Refractory Idiopathic Scleritis (CIRIS) Not yet recruiting NCT03088293 Phase 3 Infliximab;Cyclophosphamide
35 Abatacept in earLy Onset Polymyalgia Rheumatica: Study ALORS Active, not recruiting NCT03632187 Phase 3 Abatacept;Placebos
36 IMPROVE: Mycophenolate Mofetil Versus Azathioprine for Maintenance Therapy in ANCA Associated Systemic Vasculitis Terminated NCT00307645 Phase 3 Cyclophosphamide;Mycophenolate mofetil;Azathioprine;Prednisone (and methylprednisolone)
37 Prevention of Glucocorticoid-Induced Osteoporosis in Rheumatic Diseases: Alendronate Versus Alfacalcidol. Completed NCT00138983 Phase 3 Alendronate versus alfacalcidol (1-alpha OH vitamin D)
38 ASCENT-Study of Sacituzumab Govitecan in Refractory/Relapsed Triple-Negative Breast Cancer Recruiting NCT02574455 Phase 3 Sacituzumab govitecan;Eribulin;Capecitabine;Gemcitabine;Vinorelbine
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 Comparison Between a Long Term and a Conventional Maintenance Treatment With Rituximab Active, not recruiting NCT02433522 Phase 3 rituximab;Placebo
41 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
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 Safety and Efficacy Study of IFX-1 in add-on to Standard of Care in Granulomatosis With Polyangiitis (GPA) and Microscopic Polyangiitis (MPA) Recruiting NCT03712345 Phase 2 IFX-1;IFX-1;Placebo
50 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2 Gusperimus

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:

19
Lung, Skin, Kidney

MalaCards organs/tissues related to Granulomatosis with Polyangiitis:

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

Publications for Granulomatosis with Polyangiitis

Articles related to Granulomatosis with Polyangiitis:

(show top 50) (show all 1372)
# Title Authors Year
1
Flare of eosinophilic granulomatosis with polyangiitis related to pregnancy: Case report and review of the literature. ( 30456168 )
2019
2
Orbital granulomatosis with polyangiitis masquerading as invasive fungal sinusitis. ( 29303390 )
2018
3
Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRareAr cohort. ( 29943913 )
2018
4
Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis. ( 29436199 )
2018
5
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
6
Eosinophilic Granulomatosis With Polyangiitis: Newer Therapies. ( 29611001 )
2018
7
Multiple Cerebral Infarctions Due to Patent Foramen Ovale in a Patient with Eosinophilic Granulomatosis with Polyangiitis. ( 29373226 )
2018
8
Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male. ( 29366363 )
2018
9
Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis. ( 29635229 )
2018
10
Costs of Disease Relapses Among Individuals with Granulomatosis, with Polyangiitis, or Microscopic Polyangiitis in the United States. ( 29470835 )
2018
11
Letter: unlikely liver bedfellows-alpha-1 antitrypsin deficiency and granulomatosis with polyangiitis. ( 29939411 )
2018
12
Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis. ( 29908086 )
2018
13
A Case of dilated Cardiomyopathy with Eosinophilic Granulomatosis with Polyangiitis in which Active Myocardial Inflammation was Only Detected by Endomyocardial Biopsy. ( 29709930 )
2018
14
Letter: unlikely liver bedfellows-alpha-1 antitrypsin deficiency and granulomatosis with polyangiitis. Author's reply. ( 29939419 )
2018
15
Can granulomatosis with polyangiitis be diagnosed earlier in primary care? A case-control study. ( 29340693 )
2018
16
Peripheral Ulcerative Keratitis Associated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab. ( 29321417 )
2018
17
Mepolizumab Treatment of Pediatric Eosinophilic Granulomatosis With Polyangiitis. ( 29466544 )
2018
18
Myeloperoxidase-ANCA-positive granulomatosis with polyangiitis is a distinct subset of ANCA-associated vasculitis: A retrospective analysis of 455 patients from a single center in China. ( 29887327 )
2018
19
Comparison of findings on thoracic computed tomography with the severity and duration of bronchial asthma in patients with eosinophilic granulomatosis with polyangiitis. ( 29857992 )
2018
20
Eosinophilic granulomatosis with polyangiitis and laryngeal involvement: review of the literature and a cross-sectional prospective experience. ( 29888684 )
2018
21
Diffuse Alveolar Hemorrhage Developing Immediately after Immunosuppressive Treatments in a Patient with Granulomatosis with Polyangiitis Who Had Pulmonary Nodules. ( 29093401 )
2018
22
Overall Disability Sum Score for Clinical Assessment of Neurological Involvement in Eosinophilic Granulomatosis With Polyangiitis. ( 29652700 )
2018
23
A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs. ( 29560002 )
2018
24
Histopathological Findings and Increased D-Dimer Are Predictive Factors of Systemic Thromboses in Eosinophilic Granulomatosis With Polyangiitis. ( 29979194 )
2018
25
Aortic Aneurysm as a Complication of Granulomatosis with Polyangiitis Successfully Treated with Prednisolone and Cyclophosphamide: A Case Report and Review of the Literature. ( 29971178 )
2018
26
Hypocomplementemia is associated with worse renal survival in ANCA-positive granulomatosis with polyangiitis and microscopic polyangiitis. ( 29621352 )
2018
27
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
28
Polyps, grommets and eosinophilic granulomatosis with polyangiitis. ( 29310745 )
2018
29
A cohort study of comorbidity in patients with granulomatosis with polyangiitis. ( 29069513 )
2018
30
Longterm Outcomes of 188 Japanese Patients with Eosinophilic Granulomatosis with Polyangiitis. ( 29907668 )
2018
31
Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review. ( 29766394 )
2018
32
HBsAg-negative and anti-HBc-positive in eosinophilic granulomatosis with polyangiitis: a retrospective pilot study. ( 29754328 )
2018
33
Eosinophilic granulomatosis with polyangiitis: A case report. ( 29428428 )
2018
34
A case of squamous cell carcinoma of the nasal cavity in a patient with granulomatosis with polyangiitis (Wegener granulomatosis). ( 29493730 )
2018
35
Frequency, Diagnosis, Treatment, and Outcome of Gastrointestinal Disease in Granulomatosis with Polyangiitis and Microscopic Polyangiitis. ( 29419474 )
2018
36
Methylprednisolone and plasmapheresis are effective for life-threatening diffuse alveolar hemorrhage and gastrointestinal hemorrhage in granulomatosis with polyangiitis: A case report and literature review. ( 29703056 )
2018
37
Improving Mortality in End-Stage Renal Disease due to Granulomatosis with Polyangiitis from 1995 to 2014. ( 29361200 )
2018
38
Severe localised granulomatosis with polyangiitis (Wegener's granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. ( 29716529 )
2018
39
Granulomatosis With Polyangiitis Associated With Mumps Viral Infection. ( 29424761 )
2018
40
Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis. ( 29637936 )
2018
41
Eosinophilic Granulomatosis with Polyangiitis Manifested by Cholecystitis and Mononeuritis Multiplex: A Case Report. ( 29892153 )
2018
42
Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report. ( 29564565 )
2018
43
Angioimmunoblastic T-cell lymphoma mimicking eosinophilic granulomatosis with polyangiitis (Churg-Strauss). ( 29966769 )
2018
44
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) induced by immune checkpoint inhibitors. ( 29936437 )
2018
45
Development of Hypertrophic Pachymeningitis in a Patient With Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis. ( 29485545 )
2018
46
Severe/uncontrolled asthma and overall survival in atopic patients with eosinophilic granulomatosis with polyangiitis. ( 30170804 )
2018
47
Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease. ( 30057533 )
2018
48
Ophthalmic Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome): A Systematic Review of the Literature. ( 30134390 )
2018
49
Granulomatosis With Polyangiitis in a Young Adult With Down Syndrome: Therapeutic Challenges and Clues to Immunopathogenesis. ( 29200025 )
2018
50
Usefulness of Cardiac Magnetic Resonance in the Diagnosis of Löffler Endocarditis Secondary to Eosinophilic Granulomatosis with Polyangiitis. ( 30146590 )
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 9.85 BPI COL11A2 CTSG ELANE HMGB1 IL15RA
2 extracellular region GO:0005576 9.73 BPI COL11A2 CTSG ELANE FCGR3B HMGB1
3 collagen-containing extracellular matrix GO:0062023 9.65 COL11A2 CTSG ELANE PRTN3 SERPINA1
4 secretory granule GO:0030141 9.5 CTSG ELANE MPO
5 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 16)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.93 CTLA4 ETS1 HLA-DPB1 HMGB1 PTPN22 TLR9
2 response to lipopolysaccharide GO:0032496 9.78 CTSG ELANE MPO PTPN22
3 defense response to Gram-negative bacterium GO:0050829 9.73 BPI CTSG TLR9
4 defense response to bacterium GO:0042742 9.72 BPI CTSG ELANE MPO TLR9
5 immune response GO:0006955 9.56 BPI CTLA4 CTSG ETS1 FCGR3B HLA-DPB1
6 positive regulation of immune response GO:0050778 9.55 CTSG ELANE
7 positive regulation of interferon-alpha secretion GO:1902741 9.54 HMGB1 PTPN22
8 defense response to fungus GO:0050832 9.54 CTSG ELANE MPO
9 positive regulation of interleukin-8 biosynthetic process GO:0045416 9.52 ELANE TLR9
10 positive regulation of toll-like receptor 4 signaling pathway GO:0034145 9.51 HMGB1 PTPN22
11 negative regulation of interleukin-8 production GO:0032717 9.49 BPI TLR9
12 antimicrobial humoral response GO:0019730 9.46 BPI CTSG ELANE PRTN3
13 response to yeast GO:0001878 9.43 ELANE MPO
14 negative regulation of growth of symbiont in host GO:0044130 9.43 CTSG ELANE MPO
15 positive regulation of toll-like receptor 9 signaling pathway GO:0034165 9.4 HMGB1 PTPN22
16 neutrophil degranulation GO:0043312 9.23 BPI CTSG ELANE FCGR3B HMGB1 MPO

Sources for Granulomatosis with Polyangiitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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