CSS
MCID: ESN020
MIFTS: 47

Eosinophilic Granulomatosis with Polyangiitis (CSS)

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

Aliases & Classifications for Eosinophilic Granulomatosis with Polyangiitis

MalaCards integrated aliases for Eosinophilic Granulomatosis with Polyangiitis:

Name: Eosinophilic Granulomatosis with Polyangiitis 53 59 37
Churg-Strauss Syndrome 53 59 72
Granulomatous Allergic Angiitis 53 59
Egpa 53 59
Allergic Angiitis and Granulomatosis 53
Allergic Granulomatous and Angiitis 53
Churg-Strauss Vasculitis 53
Allergic Granulomatosis 53
Css 53

Characteristics:

Orphanet epidemiological data:

59
eosinophilic granulomatosis with polyangiitis
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe),<1/1000000 (Sweden),1-9/100000 (France),1-9/100000 (Sweden),<1/1000000 (United Kingdom),1-9/100000 (Norway),<1/1000000 (Spain),1-9/100000 (Australia),1-9/1000000 (Europe),1-9/100000 (Worldwide),<1/1000000 (Norway),1-9/1000000 (Germany),1-9/1000000 (Lithuania),1-9/1000000 (France),1-9/100000 (Germany),1-9/100000 (Japan); Age of onset: Adolescent,Adult,Elderly; Age of death: adolescent,adult,elderly,young Adult;

Classifications:



External Ids:

KEGG 37 H01468
MESH via Orphanet 45 C531653 D015267
ICD10 via Orphanet 34 M30.1
UMLS via Orphanet 73 C0008728
Orphanet 59 ORPHA183
UMLS 72 C0008728

Summaries for Eosinophilic Granulomatosis with Polyangiitis

KEGG : 37
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitic disorder of unknown etiology that affects small-to-medium-size blood vessels. This disease has been called Churg-Strauss syndrome (CSS) for many years, and renamed eosinophilic granulomatosis with polyangiitis (EGPA) in 2012. EGPA is characterized by asthma, hypereosinophilia, and extravascular eosinophilic granulomas. Clinically, three phases may be distinguished. The prodromal phase may persist for many years, consisting of asthma possibly associated with allergic rhinitis and often complicated by recurrent rhinosinusitis and nasal polyps. The second phase is characterized by peripheral blood eosinophilia or eosinophilic tissue infiltrates. The third phase is dominated by manifestations resulting from systemic vasculitis. Vasculitis commonly affects the skin, nerves, gastrointestinal tract, and heart. It can be serious and life-threatening. EGPA pathogenesis is not well known. The disease is probably the result of a complex interaction in which genetically and environmental factors lead to an inflammatory response whose principal players are eosinophils, T, and B lymphocytes. HLA-DRB1 and DRB4 alleles and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative.

MalaCards based summary : Eosinophilic Granulomatosis with Polyangiitis, also known as churg-strauss syndrome, is related to granulomatosis with polyangiitis and coffin-siris syndrome 1. An important gene associated with Eosinophilic Granulomatosis with Polyangiitis is FCGR3B (Fc Fragment Of IgG Receptor IIIb), and among its related pathways/superpathways is Phagosome. The drugs Cyclophosphamide and Azathioprine have been mentioned in the context of this disorder. Affiliated tissues include lung, heart and skin, and related phenotypes are weight loss and peripheral neuropathy

NIH Rare Diseases : 53 Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) is a condition characterized by asthma, high levels of eosinophils (a type of white blood cell that helps fight infection), and inflammation of small to medium sized blood vessels (vasculitis). The inflamed vessels can affect various organ systems including the lungs, gastrointestinal tract, skin, heart and nervous system. The exact cause of eosinophilic granulomatosis with polyangiitis is unknown, but it is thought to be an autoimmune disorder. Treatment may involve the use of glucocorticoids (steroids) and/or other immunosuppressive therapies. As of December 2017, mepolizumab (Nucala) became the first therapy approved specifically to treat eosinophilic granulomatosis with polyangiitis. When added to traditional steroid or immunosuppressive therapy, mepolizumab has been found to increase remission rate and time in remission for 50% of people with eosinophilic granulomatosis with polyangiitis.

Wikipedia : 75 Eosinophilic granulomatosis with polyangiitis (EGPA), also known as allergic granulomatosis, is an... more...

Related Diseases for Eosinophilic Granulomatosis with Polyangiitis

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

(show top 50) (show all 409)
# Related Disease Score Top Affiliating Genes
1 granulomatosis with polyangiitis 29.9 MPO IL27 FCGR3B
2 coffin-siris syndrome 1 12.0
3 churg-strauss syndrome 12.0
4 allergic angiitis 12.0
5 anca-associated vasculitis 11.9
6 microscopic polyangiitis 11.9
7 autoimmune lymphoproliferative syndrome 11.4
8 superficial siderosis 11.4
9 vasculitis 11.1
10 hypereosinophilic syndrome 11.0
11 granulomatous angiitis 10.9
12 neuropathy 10.7
13 peripheral nervous system disease 10.7
14 asthma-related traits 4 10.7
15 mononeuritis multiplex 10.7
16 myocarditis 10.6
17 wells syndrome 10.6
18 47,xyy 10.6
19 polyneuropathy 10.6
20 rhinitis 10.6
21 pneumonia 10.5
22 peritonitis 10.5
23 purpura 10.5
24 angina pectoris 10.5
25 maxillary sinusitis 10.5
26 acalculous cholecystitis 10.5
27 kidney disease 10.5
28 paresthesia 10.5
29 allergic rhinitis 10.5
30 glomerulonephritis 10.5
31 eosinophilic pneumonia 10.5
32 mitral valve insufficiency 10.4
33 acute kidney failure 10.4
34 exanthem 10.4
35 cardiac conduction defect 10.4
36 fibrosis of extraocular muscles, congenital, 1 10.4
37 ulcerative colitis 10.4
38 pulmonary eosinophilia 10.4
39 otitis media 10.4
40 aspergillosis 10.4
41 thrombosis 10.4
42 endocarditis 10.4
43 pericarditis 10.4
44 allergic bronchopulmonary aspergillosis 10.3
45 cholecystitis 10.3
46 ischemia 10.3
47 bronchiectasis 10.3
48 nonarteritic anterior ischemic optic neuropathy 10.3
49 myocardial infarction 10.3
50 chorea, childhood-onset, with psychomotor retardation 10.3

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



Diseases related to Eosinophilic Granulomatosis with Polyangiitis

Symptoms & Phenotypes for Eosinophilic Granulomatosis with Polyangiitis

Human phenotypes related to Eosinophilic Granulomatosis with Polyangiitis:

59 32 (show top 50) (show all 53)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 weight loss 59 32 hallmark (90%) Very frequent (99-80%) HP:0001824
2 peripheral neuropathy 59 32 hallmark (90%) Very frequent (99-80%) HP:0009830
3 sinusitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000246
4 congestive heart failure 59 32 hallmark (90%) Very frequent (99-80%) HP:0001635
5 urticaria 59 32 hallmark (90%) Very frequent (99-80%) HP:0001025
6 pulmonary infiltrates 59 32 hallmark (90%) Very frequent (99-80%) HP:0002113
7 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
8 purpura 59 32 hallmark (90%) Very frequent (99-80%) HP:0000979
9 asthma 59 32 hallmark (90%) Very frequent (99-80%) HP:0002099
10 vasculitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0002633
11 central nervous system degeneration 59 32 hallmark (90%) Very frequent (99-80%) HP:0007009
12 eosinophilia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001880
13 hypertension 59 32 frequent (33%) Frequent (79-30%) HP:0000822
14 gait disturbance 59 32 frequent (33%) Frequent (79-30%) HP:0001288
15 dysphagia 59 32 frequent (33%) Frequent (79-30%) HP:0002015
16 nausea and vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002017
17 fatigue 59 32 frequent (33%) Frequent (79-30%) HP:0012378
18 arthralgia 59 32 frequent (33%) Frequent (79-30%) HP:0002829
19 hypertrophic cardiomyopathy 59 32 frequent (33%) Frequent (79-30%) HP:0001639
20 abdominal pain 59 32 frequent (33%) Frequent (79-30%) HP:0002027
21 skin rash 59 32 frequent (33%) Frequent (79-30%) HP:0000988
22 hematuria 59 32 frequent (33%) Frequent (79-30%) HP:0000790
23 hypopigmented skin patches 59 32 frequent (33%) Frequent (79-30%) HP:0001053
24 abnormal pericardium morphology 59 32 frequent (33%) Frequent (79-30%) HP:0001697
25 abnormality of the pleura 59 32 frequent (33%) Frequent (79-30%) HP:0002103
26 venous thrombosis 59 32 frequent (33%) Frequent (79-30%) HP:0004936
27 tubulointerstitial nephritis 59 32 frequent (33%) Frequent (79-30%) HP:0001970
28 respiratory insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0002093
29 arthritis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001369
30 malabsorption 59 32 occasional (7.5%) Occasional (29-5%) HP:0002024
31 gastroesophageal reflux 59 32 occasional (7.5%) Occasional (29-5%) HP:0002020
32 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
33 proteinuria 59 32 occasional (7.5%) Occasional (29-5%) HP:0000093
34 fever 59 32 occasional (7.5%) Occasional (29-5%) HP:0001945
35 cranial nerve paralysis 59 32 occasional (7.5%) Occasional (29-5%) HP:0006824
36 subcutaneous nodule 59 32 occasional (7.5%) Occasional (29-5%) HP:0001482
37 acrocyanosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001063
38 myocardial infarction 59 32 occasional (7.5%) Occasional (29-5%) HP:0001658
39 transient ischemic attack 59 32 occasional (7.5%) Occasional (29-5%) HP:0002326
40 papule 59 32 occasional (7.5%) Occasional (29-5%) HP:0200034
41 hemiplegia/hemiparesis 59 32 occasional (7.5%) Occasional (29-5%) HP:0004374
42 myalgia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003326
43 cough 59 32 occasional (7.5%) Occasional (29-5%) HP:0012735
44 myositis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100614
45 intestinal obstruction 59 32 occasional (7.5%) Occasional (29-5%) HP:0005214
46 glomerulopathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0100820
47 hemoptysis 59 32 occasional (7.5%) Occasional (29-5%) HP:0002105
48 myocarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012819
49 endocarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100584
50 recurrent intrapulmonary hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0006535

MGI Mouse Phenotypes related to Eosinophilic Granulomatosis with Polyangiitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.13 FCGR3B IL27 MPO
2 immune system MP:0005387 8.8 FCGR3B IL27 MPO

Drugs & Therapeutics for Eosinophilic Granulomatosis with Polyangiitis

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

(show top 50) (show all 79)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
2
Azathioprine Approved Phase 4 446-86-6 2265
3
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
4
leucovorin Approved Phase 4 58-05-9 143 6006
5
Mycophenolic acid Approved Phase 4 24280-93-1 446541
6
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
7 Alkylating Agents Phase 4
8 Antineoplastic Agents, Alkylating Phase 4
9 Gastrointestinal Agents Phase 4
10 Antiemetics Phase 4
11 Neuroprotective Agents Phase 4
12 Peripheral Nervous System Agents Phase 4
13 Protective Agents Phase 4
14 Autonomic Agents Phase 4
15 Dermatologic Agents Phase 4
16 Folic Acid Antagonists Phase 4
17 Vitamin B9 Phase 4
18 Nucleic Acid Synthesis Inhibitors Phase 4
19 Vitamin B Complex Phase 4
20 Folate Phase 4
21 Anti-Infective Agents Phase 4
22 Anti-Bacterial Agents Phase 4
23 Antitubercular Agents Phase 4
24 Antibiotics, Antitubercular Phase 4
25 Antimetabolites Phase 4
26 Antimetabolites, Antineoplastic Phase 4
27
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
28
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
29
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
30
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
31
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
32
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
33 Anti-Inflammatory Agents Phase 3
34 Hormones Phase 3
35 glucocorticoids Phase 3
36 Methylprednisolone Acetate Phase 3
37 Antineoplastic Agents, Hormonal Phase 3
38 Hormone Antagonists Phase 3
39 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
40 Prednisolone acetate Phase 3
41 Pharmaceutical Solutions Phase 3
42 Antibodies Phase 3
43 Immunoglobulins Phase 3
44 Immunoglobulin G Phase 3
45 Rho(D) Immune Globulin Phase 3
46 gamma-Globulins Phase 3
47 Immunoglobulins, Intravenous Phase 3
48 Benralizumab Approved, Investigational Phase 2 1044511-01-4
49
Naltrexone Approved, Investigational, Vet_approved Phase 2 16590-41-3 5360515
50
Ethanol Approved Phase 2 64-17-5 702

Interventional clinical trials:

(show all 46)
# Name Status NCT ID Phase Drugs
1 CHUSPAN SCS BP Treatment of Churg–Strauss Syndrome Without Poor-Prognosis Factors: a Prospective Randomized Study in 72 Patients. Unknown status NCT00399399 Phase 4 azathioprine;cyclophosphamide
2 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
3 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
4 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
5 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
6 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 Recruiting NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
7 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
8 Study 200622: A Randomized, Double-blind, Placebo-controlled Study to Investigate the Efficacy and Safety of Mepolizumab in the Treatment of Adolescent and Adult Subjects With Severe Hypereosinophilic Syndrome Active, not recruiting NCT02836496 Phase 3 Mepolizumab 300 mg;Placebo matching mepolizumab;Active OCS capsules (5 mg prednisolone or prednisone);Placebo matching OCS capsules
9 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)
10 The Efficacy and Safety of Benralizumab In the Treatment of Eosinophilic Grandulomatosis With Polyangiitis (EGPA) Study: BITE Unknown status NCT03010436 Phase 2 Benralizumab
11 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
12 A Phase II, Single Center Open Label, Prospective Trial to Evaluate the Efficacy and Safety of Mepolizumab for Patients With Refractory or Relapsing Churg Strauss Syndrome Completed NCT00716651 Phase 2 mepolizumab
13 Mepolizumab As a Steroid Sparing Treatment Option in the Churg Strauss Syndrome Completed NCT00527566 Phase 1, Phase 2
14 A Phase I/II Study of the Effect of Intravenous Anti-IL-5 (Mepolizumab) SB 240563 on the Outcome and Management of Hypereosinophilic Syndromes Completed NCT00266565 Phase 1, Phase 2 Mepolizumab
15 An Open Trial of the Efficacy of Glucocorticoids and Methotrexate (MTX) in the Treatment of Systemic Vasculitis Completed NCT00001256 Phase 2 prednisone and methotrexate
16 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
17 Open-Label, to Evaluate the Efficacy and Safety of Reslizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA) Study: RITE Study Recruiting NCT02947945 Phase 2 Reslizumab
18 A Pilot Study on the Use of Rituximab in the Treatment of Churg- Strauss Syndrome With Renal Involvement Terminated NCT00424749 Phase 2 Rituximab;Prednisone
19 A Pilot Study Examining the Effect of Abatacept in ANCA Associated Vasculitis Terminated NCT00482066 Phase 2 Abatacept (Orencia)
20 High Dose Immune Suppression With Hematopoietic Stem Cell Support in Refractory Vasculitis, Necrotizing Vasculitis, Neurovascular Behcet's Disease, and Sjogren's Syndrome Terminated NCT00278512 Phase 1 Cyclophosphamide;ATG (rabbit);G-CSF;Campath-1h;Fludarabine;Tacrolimus;Mesna
21 A Qualitative Study Using Interviews With Patients Who Have Anti-neutrophil Cytoplasm Antibody (ANCA) Associated Vasculitis, to Develop a Patient Reported Outcome (PRO) Measure Unknown status NCT01729624
22 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055 anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone
23 ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis Unknown status NCT01066208
24 The ANCA Vasculitis Questionnaire (AAV-PRO©) Completed NCT02507024
25 Infliximab Versus Rituximab in Systemic Necrotizing Vasculitides With Positive ANCA After Relapse or Resistant Immunosuppressant Therapies Completed NCT00307593 Infliximab;Rituximab
26 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
27 Educational Needs of Patients With Systemic Vasculitis- an International Survey Completed NCT02190929
28 Illness Perception, Fatigue, and Function in Systemic Vasculitis (The VCRC Vasculitis Perception (VIP) Study) Completed NCT02190916
29 The Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
30 The Serum Eosinophil Cationic Protein Levels in Behçet's Disease and Its Relation to the Clinical Activity Completed NCT01584778
31 Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Recruiting NCT00315380
32 Eosinophilic Granulomatosis With Polyangiitis Cohort Recruiting NCT03036670
33 VCRC Genetic Repository One-Time DNA Protocol Recruiting NCT01241305
34 Clinical and Echocardiographic Evaluation of Patients With Granulomatosis With Polyangiitis Recruiting NCT03782870
35 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
36 VCRC Tissue Biorepository Collection Protocol Recruiting NCT02967068
37 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Recruiting NCT02006134
38 Studies of the Natural History, Pathogenesis, and Outcome of Idiopathic Systemic Vasculitis Recruiting NCT02257866
39 The Vasculitis Pregnancy Registry (V-PREG) Recruiting NCT02593565
40 Soluble CD95 Ligand Role in the Pathophysiology of ANCA Associated Vasculitis Recruiting NCT03698071
41 Single-centre Study Exploring the Utility of Hyperpolarized 129Xe Magnetic Resonance Imaging in Healthy Volunteers and Patients With Lung Disease Recruiting NCT03455686
42 NUCALA ® Subcutaneous Injection Special Drug Use Investigation (EGPA, Long-term) Active, not recruiting NCT03557060 Nucala
43 Neutrophils Function and Identification of Prognostic Factors in Granulomatosis With Polyangiitis (Formerly Named Wegener's Granulomatosis). Active, not recruiting NCT01862068
44 VCRC Validation of Patient-Reported Diagnostic Data Enrolling by invitation NCT02190942
45 Defining Immune Tolerance in ANCA-associated Vasculitis (AAV) Terminated NCT01934504
46 A Pilot Study of Immuno-ablation With Chemoimmunoradiation Followed by Autologous Hematopoietic Progenitor Cell (HPC) Transplant for Adult Subjects With Churg-Strauss Syndrome Terminated NCT02728271 Early Phase 1

Search NIH Clinical Center for Eosinophilic Granulomatosis with Polyangiitis

Genetic Tests for Eosinophilic Granulomatosis with Polyangiitis

Anatomical Context for Eosinophilic Granulomatosis with Polyangiitis

MalaCards organs/tissues related to Eosinophilic Granulomatosis with Polyangiitis:

41
Lung, Heart, Skin, Neutrophil, T Cells, Testes, Colon

Publications for Eosinophilic Granulomatosis with Polyangiitis

Articles related to Eosinophilic Granulomatosis with Polyangiitis:

(show top 50) (show all 2364)
# Title Authors PMID Year
1
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. 38 6
28514601 2017
2
Differentiation Between Eosinophilic Otitis Media and Otitis Media Associated With Eosinophilic Granulomatosis With Polyangiitis. 38
31290803 2019
3
Pruritic skin involvement of necrotizing sarcoid granulomatosis: a case report. 38
31407372 2019
4
The spectrum of therapeutic activity of mepolizumab. 38
31424304 2019
5
Mepolizumab in patients with eosinophilic granulomatosis with polyangiitis. 38
31056188 2019
6
Eosinophilic Granulomatosis with Polyangiitis: Experiences in Korean Patients. 38
31347324 2019
7
Eosinophilic granulomatosis with polyangiitis in a 10-year-old girl. 38
31427357 2019
8
A Pediatric Case of Relapsing Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab. 38
31391393 2019
9
Rapid Disappearance of Intraventricular Mobile Structures with Steroids in Eosinophilic Granulomatosis with Polyangiitis. 38
31422006 2019
10
Development of Hypertrophic Pachymeningitis in a Patient With Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis. 38
29485545 2019
11
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) induced by immune checkpoint inhibitors. 38
29936437 2019
12
New perspectives in eosinophilic granulomatosis with polyangiitis (EGPA): report of the first meeting of the European EGPA Study Group. 38
31388893 2019
13
A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease. 38
31426769 2019
14
Response to: 'Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) induced by immune checkpoint inhibitors' by Delyon et al. 38
30054370 2019
15
Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Is There Still a Role for Cyclophosphamide? 38
31277751 2019
16
Significant association between clinical characteristics and immuno-phenotypes in patients with ANCA-associated vasculitis. 38
31377799 2019
17
Epidemiology and clinical features of childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis: a clinicopathological analysis. 38
31076873 2019
18
ANCA-Associated Vasculitis: Core Curriculum 2020. 38
31358311 2019
19
Could hypereosinophilia at diagnosis estimate the current activity or predict relapse in systemic immunosuppressive drug-naïve patients with eosinophilic granulomatosis with polyangiitis? 38
31302739 2019
20
Subtypes in eosinophilic granulomatosis with polyangiitis classified according to rheumatoid factor. 38
31317422 2019
21
Co-occurrence of PLA2R-positive membranous nephropathy without crescents, and PR3-positive eosinophilic granulomatosis with polyangiitis
. 38
31347497 2019
22
Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis. 38
31146595 2019
23
Nodal Eosinophilic Granulomatosis With Polyangiitis (EGPA). 38
31356392 2019
24
A challenging diagnosis of MPO-C-ANCA EGPA. 38
31296634 2019
25
Cutaneous Vasculitis as Early Presentation of Eosinophilic Granulomatosis with Polyangiitis without Systemic Symptoms. 38
31360325 2019
26
Temporal arteritis with ultrasound halo sign in eosinophilic granulomatosis with polyangiitis. 38
31292636 2019
27
Eosinophilic granulomatosis with polyangiitis in a Nigerian woman. 38
31217209 2019
28
Causes of eosinophilic ascites - A systematic review. 38
30864403 2019
29
Update on eosinophilic granulomatosis with polyangiitis. 38
31266709 2019
30
Corneal melt secondary to eosinophilic granulomatosis with polyangiitis. 38
31229976 2019
31
The severe long-term outcomes of 'non-severe' eosinophilic granulomatosis with polyangiitis. 38
31257427 2019
32
Anti-proteinase 3-positive Eosinophilic Granulomatosis with Polyangiitis Revealed by Cardiac Tamponade: A Case Report. 38
31243203 2019
33
Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis. 38
30578883 2019
34
Anti-Neutrophil Cytoplasmic Antibodies Positivity and Anti-Leukotrienes in Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Monocentric Study on 134 Italian Patients. 38
31189169 2019
35
Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist. 38
31242923 2019
36
Targeted immunotherapy strategies in ANCA-associated vasculitis. 38
30201478 2019
37
Non-infectious aortic and mitral valve vegetations in a patient with eosinophilic granulomatosis with polyangiitis. 38
31092487 2019
38
Decrease in MPO-ANCA after administration of benralizumab in eosinophilic granulomatosis with polyangiitis. 38
31080020 2019
39
Non-severe eosinophilic granulomatosis with polyangiitis: long-term outcomes after remission-induction trial. 38
31056661 2019
40
Anti-neutrophil cytoplasmic antibody-positive eosinophilic granulomatosis with polyangiitis: can it cause membranous nephropathy? 38
30907692 2019
41
Pregnancy in a patient with eosinophilic granulomatosis with polyangiitis. 38
30634882 2019
42
Eosinophilic Granulomatosis With Polyangiitis: Histopathological Confirmation Despite Negative Serology. 38
31100285 2019
43
[Current treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)]. 38
30627842 2019
44
Refractory oral ulcers in eosinophilic granulomatosis with polyangiitis. 38
31050016 2019
45
Hepatic Microaneurysms in Eosinophilic Granulomatosis With Polyangiitis: A Clinical Image. 38
31136411 2019
46
A delayed diagnosis of eosinophilic granulomatosis with polyangiitis complicated with extensive artery occlusion of lower extremities in children: case report and literature review. 38
31138304 2019
47
[Hypereosinophilic syndrome and other rheumatic diseases with hypereosinophilia]. 38
30937528 2019
48
[Differential diagnosis of hypereosinophilia]. 38
30635705 2019
49
[Fatal cardiomyopathy in eosinophilic granulomatosis with polyangiitis]. 38
31005498 2019
50
European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides - the SHARE initiative. 38
30535249 2019

Variations for Eosinophilic Granulomatosis with Polyangiitis

Expression for Eosinophilic Granulomatosis with Polyangiitis

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

Pathways for Eosinophilic Granulomatosis with Polyangiitis

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

# Super pathways Score Top Affiliating Genes
1 10.81 MPO FCGR3B

GO Terms for Eosinophilic Granulomatosis with Polyangiitis

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 8.8 MPO IL27 FCGR3B

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

# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 8.62 MPO FCGR3B

Sources for Eosinophilic Granulomatosis with Polyangiitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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