Granulomatosis with Polyangiitis (GPA)

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 73 20 43 58 36 42 44 15 17 70
Gpa 57 20 43 58
Wegener Granulomatosis 20 13 32
Wegener Granulomatosis, Formerly 57 12
Midline Granulomatosis 20 70
Pauci-Immune Glomerulonephritis Associated with Granulomatosis with Polyangiitis 70
Wegener Granulomatosis, Formerly; Wg, Formerly 57
Necrotizing Respiratory Granulomatosis 12
Wegeners Granulomatosis 54
Wg, Formerly 57
Wg 20


Orphanet epidemiological data:

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;


granulomatosis with polyangiitis:
Onset and clinical course middle age onset pediatric onset
Inheritance polygenic inheritance


Orphanet: 58  
Rare neurological diseases
Rare circulatory system diseases
Rare renal diseases
Rare respiratory diseases
Rare systemic and rhumatological diseases

Summaries for Granulomatosis with Polyangiitis

MedlinePlus Genetics : 43 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.Another characteristic feature of GPA is the formation of granulomas, which are small areas of inflammation composed of immune cells that aid in the inflammatory reaction. The granulomas usually occur in the lungs or airways of people with this condition, although they can occur in the eyes or other organs. As granulomas grow, they can invade surrounding areas, causing tissue damage.The signs and symptoms of GPA vary based on the tissues and organs affected by vasculitis. Many people with this condition experience a vague feeling of discomfort (malaise), fever, weight loss, or other general symptoms of the body's immune reaction. In most people with GPA, inflammation begins in the vessels of the respiratory tract, leading to nasal congestion, frequent nosebleeds, shortness of breath, or coughing. Severe inflammation in the nose can lead to a hole in the tissue that separates the two nostrils (nasal septum perforation) or a collapse of the septum, causing a sunken bridge of the nose (saddle nose).The kidneys are commonly affected in people with GPA. Tissue damage caused by vasculitis in the kidneys can lead to decreased kidney function, which may cause increased blood pressure or blood in the urine, and life-threatening kidney failure. Inflammation can also occur in other regions of the body, including the eyes, middle and inner ear structures, skin, joints, nerves, heart, and brain. Depending on which systems are involved, additional symptoms can include skin rashes, inner ear pain, swollen and painful joints, and numbness or tingling in the limbs.GPA is most common in middle-aged adults, although it can occur at any age. If untreated, the condition is usually fatal within 2 years of diagnosis. Even after treatment, vasculitis can return.

MalaCards based summary : Granulomatosis with Polyangiitis, also known as gpa, is related to churg-strauss syndrome and anca-associated vasculitis, and has symptoms including angina pectoris, chest pain and edema. An important gene associated with Granulomatosis with Polyangiitis is PRTN3 (Proteinase 3), and among its related pathways/superpathways are Innate Immune System and Allograft rejection. The drugs alemtuzumab and Methotrexate have been mentioned in the context of this disorder. Affiliated tissues include lung, kidney and skin, and related phenotypes are recurrent respiratory infections and fatigue

Disease Ontology : 12 An autoimmune 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 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.

GARD : 20 Granulomatosis with polyangiitis (GPA) is a type of vasculitis or swelling (inflammation) of the blood vessels. The disease can cause swelling of the blood vessels anywhere in the body but mainly impacts the sinuses, nose, trachea (windpipe), lungs, and kidneys. The swelling can limit the flow of blood to these body parts, causing damage. Symptoms of the disease can include sinus pain, recurrent respiratory infections, joint pain, tiredness ( fatigue ), and skin lesions. The exact cause of GPA is unknown, but it is a type of autoimmune disease. Diagnosis of GPA can be made with laboratory tests such as a blood test, biopsy of affected areas, and imaging of the lungs. Treatment of GPA often includes medications such as glucocorticoids and immunosuppressants.

OMIM® : 57 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) (Updated 05-Apr-2021)

MedlinePlus : 42 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.

KEGG : 36 Granulomatosis with polyangitis (GPA) is an idiopathic, systemic inflammatory disease characterized by necrotizing granulomatous inflammation and pauci-immune small- vessel vasculitis of upper and lower respiratory tract and kidneys. The discovery of anti-neutrophil cytoplasmic antibodies (ANCAs) as a marker associated with GPA focused attention on the potential pathogenic role of these antibodies. Although ANCAs have been described that recognise a variety of myeloid antigens, only antibodies that react with proteinase 3 (Pr3) and myeloperoxidase (MPO) have consistently been linked to vasculitis syndromes. Pr3-ANCA is the predominant autoantibody found in patients with GPA. The discovery of ANCA has made the diagnosis of this disease even more possible but there is almost always a need to confirm the diagnosis via histological examination of the lesional tissue.

Wikipedia : 73 Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is an... 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 714)
# Related Disease Score Top Affiliating Genes
1 churg-strauss syndrome 33.8 PRTN3 MPO IL5
2 anca-associated vasculitis 33.0 SERPINA1 PTPN22 PRTN3 MPO HMGB1 CTLA4
3 microscopic polyangiitis 32.9 MPO FCGR3B
4 rapidly progressive glomerulonephritis 32.8 PRTN3 MPO ELANE
5 scleritis 32.7 PTPN22 PRTN3 MPO IL5 CTLA4 CCR6
7 glomerulonephritis 31.9 PRTN3 MPO ELANE CTSG
8 mononeuritis multiplex 31.8 PRTN3 MPO IL5 CD4
9 exanthem 31.6 PRTN3 CTLA4 CD4 CCR6
10 autoimmune disease 31.6 TLR9 PTPN22 PRTN3 MPO IL5 FOXP3
11 conjunctivitis 31.5 MPO IL5 FOXP3
12 dacryoadenitis 31.4 PRTN3 FOXP3 CD4 CCR6
13 crescentic glomerulonephritis 31.4 PRTN3 MPO CTSG BPI
14 orbital disease 31.4 PRTN3 CTLA4 CD4
15 lupus erythematosus 31.3 PTPN22 HMGB1 FCGR3B CTLA4
16 respiratory failure 31.3 SERPINA1 MPO IL5 ELANE CD4 CCR6
17 mononeuropathy 31.3 PRTN3 MPO CD4
18 gingivitis 31.2 MPO ELANE CTSG
19 aortitis 31.2 PRTN3 MPO CD4
20 cholangitis 31.2 PRTN3 MPO CTLA4
21 arthritis 31.1 PTPN22 HMGB1 HLA-DPB1 FOXP3 CTLA4 CCR6
22 autoimmune vasculitis 31.1 PRTN3 MPO ELANE CD4 CCR6
23 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 31.1 PTPN22 FOXP3 CTLA4
24 aspergillosis 31.1 TLR9 IL5 ELANE CCR6
25 multiple cranial nerve palsy 31.1 PRTN3 MPO
26 alpha-1-antitrypsin deficiency 31.1 SERPINA1 PRTN3 MPO ELANE
27 pneumonia 31.1 SERPINA1 MPO IL5 HMGB1 ELANE BPI
28 temporal arteritis 31.1 PTPN22 PRTN3 MPO CTLA4 CD4 CCR6
29 bone inflammation disease 31.0 PTPN22 PRTN3 FOXP3 CTLA4 CD4 CCR6
30 crohn's disease 31.0 TLR9 PTPN22 MPO FOXP3 CTLA4 CD4
31 asthma 31.0 TLR9 SERPINA1 MPO IL5 HLA-DPB1 HLA-DPA1
32 cryptogenic organizing pneumonia 31.0 PRTN3 MPO CD4
33 orbital plasma cell granuloma 31.0 PRTN3 CTLA4
34 lymphopenia 31.0 PTPN22 FOXP3 CTLA4
35 mastoiditis 30.9 PRTN3 MPO
36 folliculitis 30.9 IL5 CD4 CCR6
37 bronchiectasis 30.9 SERPINA1 MPO ELANE CTSG BPI
38 goodpasture syndrome 30.9 PRTN3 MPO CD4 CCR6
39 chickenpox 30.9 CTLA4 CD4 CD27 CCR6
40 colitis 30.9 TLR9 MPO FOXP3 CTLA4 CD4 CCR6
41 disease by infectious agent 30.8 TLR9 SERPINA1 PRTN3 CD4 CCR6
42 central nervous system vasculitis 30.8 PTPN22 PRTN3 MPO CTLA4 CD4 CCR6
43 lung disease 30.8 TLR9 SERPINA1 PRTN3 MPO IL5 HMGB1
44 diarrhea 30.8 SERPINA1 MPO IL5 FOXP3
45 dermatitis 30.8 TLR9 IL5 FOXP3 CTLA4 CD4 CCR6
46 bronchitis 30.8 SERPINA1 MPO IL5 ELANE
47 chronic conjunctivitis 30.7 IL5 CD4 CCR6
48 thyroiditis 30.7 PTPN22 FOXP3 CTLA4
49 acute frontal sinusitis 30.7 PRTN3 MPO
50 autoimmune hepatitis 30.7 HMGB1 FOXP3 CTLA4

Comorbidity relations with Granulomatosis with Polyangiitis via Phenotypic Disease Network (PDN):

Acute Kidney Failure Chronic Kidney Disease
Deficiency Anemia Neutropenia
Respiratory Failure

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:

58 31 (show top 50) (show all 86)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 recurrent respiratory infections 58 31 hallmark (90%) Very frequent (99-80%) HP:0002205
2 fatigue 58 31 hallmark (90%) Very frequent (99-80%) HP:0012378
3 fever 58 31 very rare (1%) Very frequent (99-80%) HP:0001945
4 sinusitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000246
5 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
6 hematuria 58 31 hallmark (90%) Very frequent (99-80%) HP:0000790
7 arthralgia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002829
8 glomerulopathy 58 31 hallmark (90%) Very frequent (99-80%) HP:0100820
9 cerebral ischemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002637
10 epistaxis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000421
11 weight loss 58 31 very rare (1%) Very frequent (99-80%) HP:0001824
12 vasculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002633
13 abnormal oral cavity morphology 58 31 hallmark (90%) Very frequent (99-80%) HP:0000163
14 pulmonary infiltrates 58 31 hallmark (90%) Very frequent (99-80%) HP:0002113
15 granulomatosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002955
16 nausea and vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002017
17 respiratory insufficiency 58 31 frequent (33%) Frequent (79-30%) HP:0002093
18 proteinuria 58 31 frequent (33%) Frequent (79-30%) HP:0000093
19 pulmonary fibrosis 58 31 frequent (33%) Frequent (79-30%) HP:0002206
20 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
21 hemoptysis 58 31 frequent (33%) Frequent (79-30%) HP:0002105
22 cough 58 31 frequent (33%) Frequent (79-30%) HP:0012735
23 periorbital edema 58 31 frequent (33%) Frequent (79-30%) HP:0100539
24 chest pain 58 31 frequent (33%) Frequent (79-30%) HP:0100749
25 skin rash 58 31 frequent (33%) Frequent (79-30%) HP:0000988
26 recurrent intrapulmonary hemorrhage 58 31 frequent (33%) Frequent (79-30%) HP:0006535
27 inflammatory abnormality of the eye 58 31 frequent (33%) Frequent (79-30%) HP:0100533
28 papule 58 31 frequent (33%) Frequent (79-30%) HP:0200034
29 abnormality of the hypothalamus-pituitary axis 58 31 frequent (33%) Frequent (79-30%) HP:0000864
30 elevated c-reactive protein level 58 31 frequent (33%) Frequent (79-30%) HP:0011227
31 elevated erythrocyte sedimentation rate 58 31 frequent (33%) Frequent (79-30%) HP:0003565
32 chronic pulmonary obstruction 31 frequent (33%) HP:0006510
33 hypertension 58 31 occasional (7.5%) Occasional (29-5%) HP:0000822
34 chronic otitis media 58 31 occasional (7.5%) Occasional (29-5%) HP:0000389
35 sensorineural hearing impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0000407
36 visual impairment 58 31 occasional (7.5%) Occasional (29-5%) HP:0000505
37 renal insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0000083
38 retinopathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0000488
39 cranial nerve paralysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0006824
40 venous thrombosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0004936
41 skin ulcer 58 31 occasional (7.5%) Occasional (29-5%) HP:0200042
42 diabetes insipidus 58 31 occasional (7.5%) Occasional (29-5%) HP:0000873
43 angina pectoris 58 31 occasional (7.5%) Occasional (29-5%) HP:0001681
44 myalgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003326
45 arrhythmia 58 31 occasional (7.5%) Occasional (29-5%) HP:0011675
46 hydronephrosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000126
47 proptosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000520
48 purpura 58 31 occasional (7.5%) Occasional (29-5%) HP:0000979
49 gastrointestinal hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0002239
50 sensory neuropathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0000763

Clinical features from OMIM®:

608710 (Updated 05-Apr-2021)

UMLS symptoms related to Granulomatosis with Polyangiitis:

angina pectoris; chest pain; edema; snoring; coughing

MGI Mouse Phenotypes related to Granulomatosis with Polyangiitis:

# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.8 CCR6 CD27 CD4 CTLA4 CTSG ELANE
2 immune system MP:0005387 9.47 CCR6 CD27 CD4 CTLA4 CTSG ELANE

Drugs & Therapeutics for Granulomatosis with Polyangiitis

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

(show top 50) (show all 104)
# Name Status Phase Clinical Trials Cas Number PubChem Id
alemtuzumab Approved, Investigational Phase 4 216503-57-0
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
Levoleucovorin Approved, Investigational Phase 4 68538-85-2 149436
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
Mycophenolic acid Approved Phase 4 24280-93-1 446541
Azathioprine Approved Phase 4 446-86-6 2265
Povidone Approved Phase 4 9003-39-8 131751496
Hydroxychloroquine Approved Phase 4 118-42-3 3652
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
rituximab Approved Phase 4 174722-31-7 10201696
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
18 Vitamin B9 Phase 4
19 Folic Acid Antagonists Phase 4
20 Antibiotics, Antitubercular Phase 4
21 Protective Agents Phase 4
22 Alkylating Agents Phase 4
23 Vitamin B Complex Phase 4
24 Dermatologic Agents Phase 4
25 Folate Phase 4
26 Anti-Bacterial Agents Phase 4
27 Antitubercular Agents Phase 4
28 Gastrointestinal Agents Phase 4
29 Neuroprotective Agents Phase 4
30 Antiemetics Phase 4
31 Immunosuppressive Agents Phase 4
32 Antimetabolites Phase 4
33 Anti-Infective Agents Phase 4
34 Antiparasitic Agents Phase 4
35 Antimalarials Phase 4
36 Antiprotozoal Agents Phase 4
37 Hormone Antagonists Phase 4
38 glucocorticoids Phase 4
39 Hormones Phase 4
40 Antineoplastic Agents, Hormonal Phase 4
41 Anti-Inflammatory Agents Phase 4
42 Methylprednisolone Acetate Phase 4
43 Tin Fluorides Phase 4
44 Immunologic Factors Phase 4
45 Antirheumatic Agents Phase 4
46 Antineoplastic Agents, Immunological Phase 4
Etanercept Approved, Investigational Phase 2, Phase 3 185243-69-0
Alendronate Approved Phase 3 121268-17-5, 66376-36-1 2088
Abatacept Approved Phase 3 332348-12-6 10237
Benralizumab Approved, Investigational Phase 3 1044511-01-4

Interventional clinical trials:

(show top 50) (show all 88)
# Name Status NCT ID Phase Drugs
1 Alemtuzumab for ANCA Associated Refractory Vasculitis - a Study of Safety and Efficacy Unknown status NCT01405807 Phase 4 Alemtuzumab
2 Short-Course Glucocorticoids and Rituximab in ANCA-Associated Vasculitis Completed NCT02169219 Phase 4 Glucocorticoids;Rituximab
3 Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years Comparison of Two Strategies Combining Steroids With or Without Immunosuppressants 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 MAINtenance of Remission With RITuximab Versus Azathioprine for Patients With Newly-diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. A Prospective, Randomized, Controlled, Double-blind Study: the MAINRITSEG Trial Recruiting NCT03164473 Phase 4 Rituximab;Azathioprine;Placebo-rituximab;Placebo-azathioprine
6 Hydroxychloroquine in ANCA Vasculitis Evaluation - A Multicentre, Randomised, Double-blind, Placebo-controlled Trial Recruiting NCT04316494 Phase 4 Hydroxychloroquine;Placebo
7 Low-dose Glucocorticoids Plus Rituximab Versus High-dose Glucocorticoids Plus Rituximab for Remission Induction in ANCA-associated Vasculitis; a Multicentre, Open Label, Randomised Control Trial Active, not recruiting NCT02198248 Phase 4 Rituximab;Glucocorticoids
8 Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis Terminated NCT02626845 Phase 4 Rituximab
9 Assessment of the Safety, Side Effects and Efficacy of Interventional Cryotherapy for the Eradication of Benign Airway Disease("ICE THE BAD") Terminated NCT00747461 Phase 4
10 Comparative Study of the Efficacy of Induction Therapy With Cyclophosphamide or Mycophenolate Mofetil for Non-Life-Threatening Relapses of PR3- or MPO-ANCA Associated Vasculitis Unknown status NCT00103792 Phase 3 mycophenolate mofetil;cyclophosphamide
11 Treatment of ANCA-Associated Vasculitides : Corticosteroids and Pulse Cyclophosphamide Followed by Maintenance Therapy With Methotrexate or Azathioprine: a Prospective Multicenter Randomized Trial 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
12 Evaluation of Rituximab-based Regimen Compared to Conventional Therapeutic Strategy For Remission Induction In Patients With Newly-Diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. Prospective, Randomized, Controlled, Double-blind Study Unknown status NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
13 MAINtenance of Remission Using RITuximab in Systemic ANCA-associated Vasculitis Completed NCT00748644 Phase 3 Rituximab;Azathioprine
14 Wegener's Granulomatosis Etanercept Trial (WGET) Completed NCT00005007 Phase 2, Phase 3 Etanercept
15 A Phase 3, Multi-Center, Multinational, Randomized, Double-Blind, Study to Evaluate the Efficacy and Safety of Belimumab (HGS1006) in Combination With Azathioprine for the Maintenance of Remission in Wegener's Granulomatosis and Microscopic Polyangiitis Completed NCT01663623 Phase 3 Azathioprine
16 Plasma Exchange and Glucocorticoid Dosing in the Treatment of Anti-neutrophil Cytoplasm Antibody Associated Vasculitis: an International Randomized Controlled Trial Completed NCT00987389 Phase 3 Glucocorticoids [Standard Dose];Glucocorticoids [Reduced Dose]
17 MAINtenance of Remission Using RITuximab in Systemic ANCA-associated Vasculitis II Completed NCT01731561 Phase 3 Rituximab (Arm B);Rituximab (Arm A)
18 Randomized Trial of Intravenous Pulse Versus Oral Continuous Cyclophosphamide for Induction of Remission in Systemic ANCA-Associated Vasculitides Completed NCT00430105 Phase 2, Phase 3 cyclophosphamide
19 Prevention of Glucocorticoid-Induced Osteoporosis in Patients With Rheumatic Diseases. The STOP-Study: a Randomized Placebo Controlled Trial With Alendronate Versus Alfacalcidol. Completed NCT00138983 Phase 3 Alendronate versus alfacalcidol (1-alpha OH vitamin D)
20 Rituximab Therapy for the Induction of Remission and Tolerance in ANCA-Associated Vasculitis (ITN021AI) Completed NCT00104299 Phase 2, Phase 3 Rituximab plus cyclophosphamide placebo (rituximab group);Cyclophosphamide plus rituximab placebo (control group);Azathioprine;Methylprednisolone (or other glucocorticoid);Prednisone
21 Evaluation of a New Treatment Strategy for Patients With Microscopic Polyangiitis, Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome) Without Poor Prognosis Factors Completed NCT00647166 Phase 3 corticosteroid and azathioprine;corticosteroid and placebo
22 An International, Open Label, Randomised Controlled Trial Comparing Rituximab With Azathioprine as Maintenance Therapy in Relapsing ANCA-associated Vasculitis Completed NCT01697267 Phase 3 Azathioprine
23 A Double-blind, Randomised, Placebo-controlled Study to Investigate the Efficacy and Safety of Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis in Subjects Receiving Standard of Care Therapy Completed NCT02020889 Phase 3 Placebo
24 Abatacept (CTLA4-Ig) for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's) (ABROGATE) Recruiting NCT02108860 Phase 3 Abatacept;placebo
25 The Assessment of Prednisone In Remission Trial (TAPIR) - Centers of Excellence Approach Recruiting NCT01940094 Phase 3 5 mg Prednisone;0 mg Prednisone
26 A Randomized, Double-blind, Active-controlled 52-week Study With an Open-label Extension to Evaluate the Efficacy and Safety of Benralizumab Compared to Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA) in Patients Receiving Standard of Care Therapy Recruiting NCT04157348 Phase 3
27 The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach Active, not recruiting NCT01933724 Phase 3 5 mg prednisone;0 mg prednisone
28 Mepolizumab Long-term Access Programme for Subjects Who Participated in Study MEA115921 (Placebo-controlled Study of Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis in Subjects Receiving Standard-of-care Therapy) Active, not recruiting NCT03298061 Phase 3 Mepolizumab;Prednisolone
29 Randomised, Evaluator-Blinded, Multicentre, International, Parallel-Group, Active-Controlled Clinical Trial of Gusperimus Versus Conventional Therapy in Relapse of Granulomatosis With Polyangiitis (Wegener's Granulomatosis) SPARROW Study - SPAnidin in Relapsing GRanulomatosis With POlyangiitis Wegener's Granulomatosis) Terminated NCT01446211 Phase 3 Gusperimus + glucocorticoids;cyclophosphamide followed by methotrexate (azathioprine) + glucocorticoids or methotrexate (azathioprine) + glucocorticoids
30 Randomised Trial of Plasma Exchange or High Dose Methyl Prednisolone as Adjunctive Therapy for Severe Renal Vasculitis Terminated NCT01408836 Phase 2, Phase 3 Intravenous methyl prednisolone;Methyl prednisolone
31 Intravenous Immunoglobulin After Relapse in Vasculitis (Microscopic Polyangiitis, Wegener's Granulomatosis and SHURG-STRAUSS Syndrome) During and After Corticosteroids and Immunosuppressant Therapies a Multicenter Prospective Trial Terminated NCT00307658 Phase 3 Intravenous immunoglobulins (human immunoglobulins G)
32 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
33 Open-Label, to Evaluate the Efficacy and Safety of Reslizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA) Study: RITE Study Unknown status NCT02947945 Phase 2 Reslizumab
34 A Randomized Trial Examining the Use of Daclizumab in Wegener's Granulomatosis Completed NCT00040248 Phase 2 Daclizumab
35 A Multi-Center, Open-label Pilot Study of Abatacept (CTLA4-Ig) in the Treatment of Mild Relapsing Wegener's Granulomatosis Completed NCT00468208 Phase 1, Phase 2 Abatacept
36 Phase I/II Trial of TNFR:Fc (Etanercept) in Patients With Wegener's Granulomatosis Completed NCT00001901 Phase 2 Etanercept
37 A Randomized Trial Comparing Methotrexate Versus Mycophenolate Mofetil for Remission Maintenance in Wegener's Granulomatosis and Related Vasculitides Completed NCT00004567 Phase 2 Mycophenolate Mofetil
38 Treatment of Wegener's Granulomatosis With Cyclophosphamide Completed NCT00001155 Phase 2 cyclophosphamide
39 Phase II Study on Gusperimus in Patients With Refractory Wegener's Granulomatosis Completed NCT00530075 Phase 2 Gusperimus
40 Phase II Pilot Cohort Study to Investigate the Safety and Efficacy of Infliximab as Additional Therapy in the Treatment if Anti-Neutrophil Cytoplasm Antibody Associated Vasculitis Completed NCT00753103 Phase 2 Cyclophosphamide;Prednisolone;Azathioprine;Mycophenolate mofetil;Methylprednisolone
41 A Phase IIA, International, Multicenter, Open-label, Uncontrolled Study to Evaluate The Safety And Pharmacokinetics of 4 × 375 mg/m2 Intravenous Rituximab in Pediatric Patients With Severe Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis Completed NCT01750697 Phase 2 Rituximab
42 An Open Trial of the Efficacy of Glucocorticoids and Methotrexate (MTX) in the Treatment of Systemic Vasculitis Completed NCT00001256 Phase 2 prednisone and methotrexate
43 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
44 A Randomised, Double Blind, Controlled Mechanistic Study of Rituximab and Belimumab Combination Therapy in PR3 ANCA-associated Vasculitis Recruiting NCT03967925 Phase 2 Belimumab;Rituximab;Prednisolone
45 Trimethoprim-Sulfamethoxazole Effects on the Nasal Microbiome in Granulomatosis With Polyangiitis Active, not recruiting NCT03919435 Phase 1, Phase 2 Trimethoprim Sulfamethoxazole
46 A Randomized, Double-blind, Double-dummy, Active-controlled, Multicenter, 2-part Phase II Study on Replacement of Steroids by IFX-1 in Active Granulomatosis With Polyangiitis (GPA) and Microscopic Polyangiitis (MPA) Active, not recruiting NCT03895801 Phase 2 IFX-1;Placebo-IFX-1;Glucocorticoid (GC);Placebo-Glucocorticoid (Placebo-GC)
47 Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Phase II Efficacy and Safety Study of IFX-1 in Add-On to Standard of Care in Granulomatosis With Polyangiitis (GPA) and Microscopic Polyangiitis (MPA) Active, not recruiting NCT03712345 Phase 2 IFX-1;IFX-1;Placebo
48 Induction of Regulatory t Cells by Low Dose IL2 in Autoimmune and Inflammatory Diseases: a Transnosographic Approach Active, not recruiting NCT01988506 Phase 2 Interleukin 2
49 A Randomized, Double-Blind, Placebo-Controlled Trial to Evaluate the Efficacy, Safety, and Tolerability of Blisibimod in Addition to Methotrexate During Induction of Remission in Subjects With ANCA-Associated Small Vessel Vasculitis Withdrawn NCT01598857 Phase 2 Blisibimod;Placebo
50 Mycophenolate Mofetil in the Treatment of Wegener's Granulomatosis and Related Vasculitides Completed NCT00001764 Phase 1 Mycophenolate Mofetil

Search NIH Clinical Center for Granulomatosis with Polyangiitis

Inferred drug relations via UMLS 70 / NDF-RT 51 :


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:

Lung, Kidney, Skin

MalaCards organs/tissues related to Granulomatosis with Polyangiitis:

Neutrophil, Kidney, Trachea, Eye, Lung, Pituitary, Monocytes

Publications for Granulomatosis with Polyangiitis

Articles related to Granulomatosis with Polyangiitis:

(show top 50) (show all 3145)
# Title Authors PMID Year
Genetically distinct subsets within ANCA-associated vasculitis. 61 57
22808956 2012
Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. 61 57
21374588 2011
Clinical characteristics and prognosis in 269 patients with antineutrophil cytoplasimc antibody associated vasculitis. 42 61
33053532 2020
Rituximab Biosimilar Prevents Poor Outcomes of Microscopic Polyangiitis and Granulomatosis with Polyangiitis as Effectively as Rituximab Originator. 42 61
32734735 2020
Mycophenolic acid and 6-mercaptopurine both inhibit B-cell proliferation in granulomatosis with polyangiitis patients, whereas only mycophenolic acid inhibits B-cell IL-6 production. 61 42
32645052 2020
New genomic region for Wegener's granulomatosis as revealed by an extended association screen with 202 apoptosis-related genes. 57
14968360 2004
Catalytic activity and inhibition of wegener antigen proteinase 3 on the cell surface of human polymorphonuclear neutrophils. 54 61
19447886 2009
A hydrophobic patch on proteinase 3, the target of autoantibodies in Wegener granulomatosis, mediates membrane binding via NB1 receptors. 54 61
18854317 2008
Wegener's granulomatosis presenting as pyoderma gangrenosum. 61 54
18076660 2008
Association study of Wegener granulomatosis and the functionally relevant A645G polymorphism in the bactericidal/permeability increasing protein (BPI) gene. 61 54
15686586 2005
The influence of different cultivating conditions on polymorphonuclear leukocyte apoptotic process in vitro, II: ultrastructural characteristics of PMN populations incubated with proteinase 3 anti-neutrophil autoantibodies. 54 61
15931779 2005
Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo. 61 54
15150076 2004
Membrane expression of proteinase 3 is genetically determined. 54 61
12506139 2003
Genomic organization and chromosomal localization of mouse proteinase 3 (Myeloblastin). 54 61
10051312 1999
Structural analysis of human antibodies to proteinase 3 from patients with Wegener granulomatosis. 61 54
9218586 1997
A proposed role of neutrophil extracellular traps and their interplay with fibroblasts in ANCA-associated vasculitis lung fibrosis. 61
33609801 2021
Otologic Manifestations of Eosinophilic Granulomatosis With Polyangiitis: A Systematic Review. 61
33534386 2021
Prevalence of Eosinophilic Granulomatosis With Polyangiitis and Associated Health Care Utilization Among Patients With Concomitant Asthma in US Commercial Claims Database. 61
31693654 2021
Endobronchial vasculitis in childhood granulomatosis with polyangiitis. 61
33680472 2021
A case of urethrovaginal fistula caused by granulomatosis with polyangiitis mimicking malignancy. 61
33792141 2021
Multiple facial ulcers as a presentation of localized granulomatosis with polyangiitis associated with IgG4-related disease. 61
33259062 2021
Sustained Remission of Granulomatosis With Polyangiitis After Discontinuation of Glucocorticoids and Immunosuppressant Therapy: Data From the French Vasculitis Study Group Registry. 61
33029946 2021
[Acute posterior multifocal placoid pigment epitheliopathy and granulomatosis with polyangiitis: A case report]. 61
33494975 2021
Early add-on administration of mepolizumab and intravenous immunoglobulin effective in treating eosinophilic granulomatosis with polyangiitis. 61
33305381 2021
Successful Treatment with Rituximab for Granulomatosis with Polyangiitis and Multiple Cranial Neuropathies. 61
33132328 2021
[The 487th case: prominent eyes, headache, blurred vision]. 61
33765713 2021
Eosinophilic Granulomatosis With Polyangiitis: An Unusual Case of Pediatric Subglottic Stenosis. 61
32562500 2021
Granulomatosis with polyangiitis presenting as pancreatic disease. 61
33653864 2021
Effectiveness and safety of mepolizumab in combination with corticosteroids in patients with eosinophilic granulomatosis with polyangiitis. 61
33726827 2021
Granulomatosis With Polyangiitis and Microscopic Polyangiitis: A Systematic Review and Meta-Analysis of Benefits and Harms of Common Treatments. 61
33590973 2021
Granulomatosis with polyangiitis complicated by genital involvement: sustained response to rituximab. 61
33738952 2021
Comment on: "Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)". 61
33682889 2021
Strawberry gums as an initial presentation of severe granulomatosis with polyangiitis. 61
32929497 2021
Paravertebral Pseudotumour in Granulomatosis with Polyangiitis. 61
31526658 2021
Benralizumab as a Steroid-Sparing Treatment Option in Eosinophilic Granulomatosis with Polyangiitis. 61
33065367 2021
Lessons of the month: ANCA-associated vasculitis - granulomatosis with polyangiitis: 'the great mimic'. 61
33762392 2021
Isolated CNS involvement in eosinophilic granulomatosis with polyangiitis treated with mepolizumab: A case report. 61
33773378 2021
Dynamic Changes in the Nasal Microbiome Associated with Disease Activity in Patients with Granulomatosis with Polyangiitis. 61
33682371 2021
Cardiac involvement in eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): Prospective evaluation at a tertiary referral centre. 61
33358337 2021
Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China. 61
33789679 2021
Granulomatosis with polyangiitis manifesting as fever of unknown origin. 61
33771775 2021
Granulomatosis with polyangiitis presenting as a solitary renal mass: A case report with imaging and literature review. 61
33510827 2021
Eosinophil ETosis-mediated release of galectin-10 in eosinophilic granulomatosis with polyangiitis. 61
33750029 2021
Granulomatosis with Polyangiitis Masquerading as Pituitary Adenoma with Apoplexy. 61
33784948 2021
Sequence-Based Screening of Patients With Idiopathic Polyarteritis Nodosa, Granulomatosis With Polyangiitis, and Microscopic Polyangiitis for Deleterious Genetic Variants in ADA2. 61
33021335 2021
Vascular endothelial growth factor (VEGF)-A and VEGF-A165b are associated with time to remission of granulomatosis with polyangiitis in a nationwide Japanese prospective cohort study. 61
33081494 2021
Cardiac magnetic resonance imaging for the detection of myocardial involvement in granulomatosis with polyangiitis. 61
33057879 2021
Gingival Swelling as the Initial Manifestation of Granulomatosis with Polyangiitis. 61
32096099 2021
Acute pancreatitis: an atypical presentation of granulomatosis with polyangiitis. 61
32901286 2021
Head and neck manifestations of granulomatosis with polyangiitis. 61
33237805 2021

Variations for Granulomatosis with Polyangiitis

Copy number variations for Granulomatosis with Polyangiitis from CNVD:

# CNVD ID Chromosome 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

Pathways related to Granulomatosis with Polyangiitis according to GeneCards Suite gene sharing:

(show all 22)
# Super pathways Score Top Affiliating Genes
Show member pathways
Show member pathways
Show member pathways
Show member pathways
Show member pathways
Show member pathways
Show member pathways
13 11.74 HLA-DPB1 HLA-DPA1 CTLA4
14 11.7 IL5 HLA-DPB1 HLA-DPA1 CD4
15 11.67 IL5 CD4 CCR6
Show member pathways
11.64 IL5 FOXP3 CTLA4
Show member pathways
18 11.56 PRTN3 MPO ELANE CD4
19 11.47 PTPN22 HLA-DPA1 CTLA4 CD4
21 11.38 IL5 FOXP3 CD4

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 region GO:0005576 9.9 TLR9 SERPINA1 PRTN3 MPO IL5 HMGB1
2 extracellular space GO:0005615 9.86 SERPINA1 PRTN3 MPO IL5 HMGB1 ELANE
3 cell surface GO:0009986 9.56 HMGB1 HLA-DPB1 HLA-DPA1 ELANE CTSG CD4
4 azurophil granule lumen GO:0035578 9.02 PRTN3 MPO ELANE CTSG BPI

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

(show all 29)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 10.07 TLR9 PTPN22 HMGB1 HLA-DPB1 HLA-DPA1 CTLA4
2 adaptive immune response GO:0002250 10 HMGB1 HLA-DPB1 HLA-DPA1 CTLA4 CD4
3 defense response to bacterium GO:0042742 9.91 TLR9 MPO ELANE CTSG BPI
4 positive regulation of JNK cascade GO:0046330 9.8 TLR9 HMGB1 CD27
5 response to lipopolysaccharide GO:0032496 9.8 PTPN22 MPO FOXP3 ELANE CTSG
6 negative regulation of tumor necrosis factor production GO:0032720 9.79 PTPN22 FOXP3 BPI
7 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.77 TLR9 HMGB1 CD27
8 positive regulation of T cell proliferation GO:0042102 9.77 HLA-DPB1 HLA-DPA1 CD4
9 positive regulation of interleukin-8 production GO:0032757 9.76 TLR9 HMGB1 ELANE
10 defense response to Gram-negative bacterium GO:0050829 9.76 TLR9 CTSG CD4 BPI
11 negative regulation of interleukin-6 production GO:0032715 9.73 TLR9 PTPN22 FOXP3 BPI
12 positive regulation of interferon-beta production GO:0032728 9.72 TLR9 PTPN22 HMGB1
13 defense response to fungus GO:0050832 9.71 MPO ELANE CTSG
14 positive regulation of T cell activation GO:0050870 9.67 HLA-DPB1 HLA-DPA1 CD4
15 positive regulation of interferon-gamma production GO:0032729 9.67 TLR9 PTPN22 HLA-DPB1 HLA-DPA1
16 positive regulation of interferon-alpha production GO:0032727 9.65 TLR9 PTPN22 HMGB1
17 negative regulation of immune response GO:0050777 9.64 FOXP3 CTLA4
18 leukocyte migration involved in inflammatory response GO:0002523 9.64 ELANE CCR6
19 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.63 HLA-DPB1 HLA-DPA1
20 positive regulation of immune response GO:0050778 9.63 ELANE CTSG
21 T cell receptor signaling pathway GO:0050852 9.63 PTPN22 HLA-DPB1 HLA-DPA1 FOXP3 CTLA4 CD4
22 positive regulation of toll-like receptor 4 signaling pathway GO:0034145 9.62 PTPN22 HMGB1
23 antimicrobial humoral response GO:0019730 9.62 PRTN3 ELANE CTSG BPI
24 positive regulation of toll-like receptor 9 signaling pathway GO:0034165 9.58 PTPN22 HMGB1
25 tumor necrosis factor production GO:0032640 9.57 TLR9 HMGB1
26 response to yeast GO:0001878 9.56 MPO ELANE
27 negative regulation of interleukin-8 production GO:0032717 9.56 TLR9 PTPN22 ELANE BPI
28 neutrophil degranulation GO:0043312 9.56 SERPINA1 PRTN3 MPO HMGB1 FCGR3B ELANE
29 immune response GO:0006955 9.32 TLR9 IL5 HLA-DPB1 HLA-DPA1 FCGR3B CTSG

Molecular functions related to Granulomatosis with Polyangiitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transmembrane signaling receptor activity GO:0004888 8.92 TLR9 FCGR3B CD4 CD27

Sources for Granulomatosis with Polyangiitis

9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
31 HPO
32 ICD10
33 ICD10 via Orphanet
37 LifeMap
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
69 Tocris
71 UMLS via Orphanet
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