CSS
MCID: CHR001
MIFTS: 60

Churg-Strauss Syndrome (CSS)

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

Aliases & Classifications for Churg-Strauss Syndrome

MalaCards integrated aliases for Churg-Strauss Syndrome:

Name: Churg-Strauss Syndrome 12 74 52 58 54 43 15 17 71
Eosinophilic Granulomatosis with Polyangiitis 52 58 36
Granulomatous Allergic Angiitis 52 58
Churg-Strauss Vasculitis 12 52
Egpa 52 58
Allergic Angiitis and Granulomatosis 52
Allergic Granulomatous and Angiitis 52
Allergic Granulomatosis Angiitis 12
Allergic Granulomatous Angiitis 12
Allergic Granulomatosis 52
Css 52

Characteristics:

Orphanet epidemiological data:

58
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:

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


Summaries for Churg-Strauss Syndrome

KEGG : 36 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 : Churg-Strauss Syndrome, also known as eosinophilic granulomatosis with polyangiitis, is related to microscopic polyangiitis and mononeuritis multiplex, and has symptoms including asthma An important gene associated with Churg-Strauss Syndrome is PRTN3 (Proteinase 3), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Cyclophosphamide and Azathioprine have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and skin, and related phenotypes are weight loss and peripheral neuropathy

Disease Ontology : 12 A vasculitits that is systemic vasculitis realized as blood vessel inflammation and has symptom asthma along with hay fever, rash and gastrointestinal bleeding.

NIH Rare Diseases : 52 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 : 74 Eosinophilic granulomatosis with polyangiitis (EGPA), also known as allergic granulomatosis, is an... more...

Related Diseases for Churg-Strauss Syndrome

Diseases related to Churg-Strauss Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 746)
# Related Disease Score Top Affiliating Genes
1 microscopic polyangiitis 33.7 TNF MPO
2 mononeuritis multiplex 32.1 PRTN3 MPO CD40LG
3 granulomatous angiitis 32.0 PRTN3 IL5
4 autoimmune lymphoproliferative syndrome 31.8 TNF IL2 IL10 CD40LG
5 vasculitis 31.7 TNF THBD PRTN3 MPO IL17A CD40LG
6 rapidly progressive glomerulonephritis 31.5 PRTN3 MPO CD40LG
7 purpura 31.4 TNF THBD IL10 CD40LG
8 chronic eosinophilic pneumonia 31.4 RNASE3 IL5 CCL11
9 mononeuropathy 31.4 TNF PRTN3 MPO CD40LG
10 ige responsiveness, atopic 31.3 RNASE3 IL5 IL10
11 chronic rhinitis 31.2 RNASE3 MPO IL5
12 anca-associated vasculitis 31.2 PRTN3 MPO HMGB1
13 pericarditis 31.2 TNF CXCL8 CD40LG
14 maxillary sinusitis 31.2 RNASE3 PRTN3 MPO CD40LG
15 hypereosinophilic syndrome, idiopathic 31.1 IL5 IL3
16 allergic bronchopulmonary aspergillosis 31.1 IL5 IL10 CCL17
17 orbital plasma cell granuloma 31.1 PRTN3 CD40LG
18 neuritis 31.1 TNF IL10 CXCL8
19 scleritis 30.9 TNF PRTN3 IL5
20 eosinophilic gastritis 30.9 IL5 CCL26 CCL11
21 endomyocardial fibrosis 30.8 TNF RNASE3 PRG2 IL5 IL10
22 multifocal choroiditis 30.8 TNF IL10
23 wells syndrome 30.7 RNASE3 IL5 IL2 CXCL8
24 cryoglobulinemia 30.7 TNF IL10 CD40LG
25 hypersensitivity vasculitis 30.7 PRTN3 MPO CD40LG
26 myocarditis 30.7 TNF IL2 IL17A IL10 CD40LG
27 goodpasture syndrome 30.7 PRTN3 MPO CD40LG
28 proctitis 30.7 TNF RNASE3 MPO
29 eosinophilic pneumonia 30.7 RNASE3 IL5 IL3 CXCL8 CCL17 CCL11
30 pulmonary embolism 30.6 THBD MPO CD40LG
31 autoimmune vasculitis 30.6 TNF PRTN3 MPO IL2
32 temporal arteritis 30.6 TNF PRTN3 MPO IL17A CD40LG
33 optic neuritis 30.6 TNF IL10 CXCL8
34 polyradiculoneuropathy 30.6 IL17A IL10 CD40LG
35 choroiditis 30.6 TNF IL10 CD40LG
36 henoch-schoenlein purpura 30.6 RNASE3 PRTN3 MPO CD40LG
37 endocarditis 30.6 TNF IL17A IL10 CXCL8
38 ileus 30.5 TNF IL17A IL10
39 cellulitis 30.5 TNF RNASE3 IL5 IL10 CD40LG
40 retinal vein occlusion 30.5 TNF CXCL8 CD40LG
41 cryptococcosis 30.5 TNF IL17A IL10
42 loeffler syndrome 30.5 PRG2 IL5 CD40LG
43 iridocyclitis 30.4 TNF IL17A CD40LG
44 stevens-johnson syndrome/toxic epidermal necrolysis 30.4 IL5 IL2 CCL17
45 aspergillosis 30.4 TNF IL5 IL10 CXCL8 CCL17
46 eosinophilic gastroenteritis 30.3 RNASE3 PRG2 IL5 IL3 CCL26 CCL11
47 toxocariasis 30.3 RNASE3 IL5 CD40LG
48 thyroiditis 30.3 TNF IL2 IL10
49 peripheral nervous system disease 30.3 TNF IL2 IL17A IL10 CXCL8 CD40LG
50 spondylitis 30.3 TNF IL17A IL10

Graphical network of the top 20 diseases related to Churg-Strauss Syndrome:



Diseases related to Churg-Strauss Syndrome

Symptoms & Phenotypes for Churg-Strauss Syndrome

Human phenotypes related to Churg-Strauss Syndrome:

58 31 (show top 50) (show all 53)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 weight loss 58 31 hallmark (90%) Very frequent (99-80%) HP:0001824
2 peripheral neuropathy 58 31 hallmark (90%) Very frequent (99-80%) HP:0009830
3 sinusitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000246
4 congestive heart failure 58 31 hallmark (90%) Very frequent (99-80%) HP:0001635
5 urticaria 58 31 hallmark (90%) Very frequent (99-80%) HP:0001025
6 pulmonary infiltrates 58 31 hallmark (90%) Very frequent (99-80%) HP:0002113
7 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
8 purpura 58 31 hallmark (90%) Very frequent (99-80%) HP:0000979
9 asthma 58 31 hallmark (90%) Very frequent (99-80%) HP:0002099
10 vasculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002633
11 central nervous system degeneration 58 31 hallmark (90%) Very frequent (99-80%) HP:0007009
12 eosinophilia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001880
13 nausea and vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002017
14 gait disturbance 58 31 frequent (33%) Frequent (79-30%) HP:0001288
15 dysphagia 58 31 frequent (33%) Frequent (79-30%) HP:0002015
16 hypertension 58 31 frequent (33%) Frequent (79-30%) HP:0000822
17 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
18 arthralgia 58 31 frequent (33%) Frequent (79-30%) HP:0002829
19 hypertrophic cardiomyopathy 58 31 frequent (33%) Frequent (79-30%) HP:0001639
20 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
21 skin rash 58 31 frequent (33%) Frequent (79-30%) HP:0000988
22 hematuria 58 31 frequent (33%) Frequent (79-30%) HP:0000790
23 hypopigmented skin patches 58 31 frequent (33%) Frequent (79-30%) HP:0001053
24 abnormal pericardium morphology 58 31 frequent (33%) Frequent (79-30%) HP:0001697
25 abnormality of the pleura 58 31 frequent (33%) Frequent (79-30%) HP:0002103
26 venous thrombosis 58 31 frequent (33%) Frequent (79-30%) HP:0004936
27 tubulointerstitial nephritis 58 31 frequent (33%) Frequent (79-30%) HP:0001970
28 respiratory insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0002093
29 arthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001369
30 malabsorption 58 31 occasional (7.5%) Occasional (29-5%) HP:0002024
31 gastroesophageal reflux 58 31 occasional (7.5%) Occasional (29-5%) HP:0002020
32 proteinuria 58 31 occasional (7.5%) Occasional (29-5%) HP:0000093
33 renal insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0000083
34 fever 58 31 occasional (7.5%) Occasional (29-5%) HP:0001945
35 cranial nerve paralysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0006824
36 subcutaneous nodule 58 31 occasional (7.5%) Occasional (29-5%) HP:0001482
37 acrocyanosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001063
38 myocardial infarction 58 31 occasional (7.5%) Occasional (29-5%) HP:0001658
39 transient ischemic attack 58 31 occasional (7.5%) Occasional (29-5%) HP:0002326
40 papule 58 31 occasional (7.5%) Occasional (29-5%) HP:0200034
41 hemiplegia/hemiparesis 58 31 occasional (7.5%) Occasional (29-5%) HP:0004374
42 myalgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003326
43 cough 58 31 occasional (7.5%) Occasional (29-5%) HP:0012735
44 myositis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100614
45 intestinal obstruction 58 31 occasional (7.5%) Occasional (29-5%) HP:0005214
46 glomerulopathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0100820
47 hemoptysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002105
48 myocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012819
49 endocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100584
50 recurrent intrapulmonary hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0006535

Symptoms:

12
  • asthma

GenomeRNAi Phenotypes related to Churg-Strauss Syndrome according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.77 CD40LG CXCL8 IL10 IL17A IL2 MPO
2 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.77 CD40LG CXCL8 IL10 IL17A IL2 MPO

MGI Mouse Phenotypes related to Churg-Strauss Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.93 CCL11 CCL17 CD40LG IL10 IL17A IL2
2 immune system MP:0005387 9.77 CCL11 CCL17 CD40LG CYSLTR1 IL10 IL17A
3 respiratory system MP:0005388 9.28 CCL11 CYSLTR1 IL10 IL17A IL2 IL5

Drugs & Therapeutics for Churg-Strauss Syndrome

Drugs for Churg-Strauss Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 74)
# 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
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
5
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
6 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
7
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
8
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
9
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
10
leucovorin Approved Phase 4 58-05-9 6006 143
11
Mycophenolic acid Approved Phase 4 24280-93-1 446541
12
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
13
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
14 Alkylating Agents Phase 4
15 Gastrointestinal Agents Phase 4
16 Antiemetics Phase 4
17 Methylprednisolone Acetate Phase 4
18 Anti-Inflammatory Agents Phase 4
19 Hormone Antagonists Phase 4
20 Neuroprotective Agents Phase 4
21 Protective Agents Phase 4
22 Autonomic Agents Phase 4
23 Antineoplastic Agents, Hormonal Phase 4
24 glucocorticoids Phase 4
25 Hormones Phase 4
26 Dermatologic Agents Phase 4
27 Folic Acid Antagonists Phase 4
28 Vitamin B Complex Phase 4
29 Vitamin B9 Phase 4
30 Folate Phase 4
31 Anti-Infective Agents Phase 4
32 Antitubercular Agents Phase 4
33 Antibiotics, Antitubercular Phase 4
34 Anti-Bacterial Agents Phase 4
35 Antimetabolites Phase 4
36 Benralizumab Approved, Investigational Phase 3 1044511-01-4
37 Respiratory System Agents Phase 3
38 Anti-Asthmatic Agents Phase 3
39 Pharmaceutical Solutions Phase 3
40 Immunoglobulins Phase 3
41 Antibodies Phase 3
42 Immunoglobulin G Phase 3
43 Rho(D) Immune Globulin Phase 3
44 Immunoglobulins, Intravenous Phase 3
45 gamma-Globulins Phase 3
46 Reslizumab Approved, Investigational Phase 2
47
Naltrexone Approved, Investigational, Vet_approved Phase 2 16590-41-3 5360515
48
Ethanol Approved Phase 2 64-17-5 702
49
rituximab Approved Phase 2 174722-31-7 10201696
50
Abatacept Approved Phase 2 332348-12-6 10237

Interventional clinical trials:

(show all 47)
# 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 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 Completed NCT02836496 Phase 3 Mepolizumab 300 mg;Placebo matching mepolizumab;Active OCS capsules (5 mg prednisolone or prednisone);Placebo matching OCS capsules
7 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
8 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
9 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
10 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)
11 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
12 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
13 The Efficacy and Safety of Benralizumab In the Treatment of Eosinophilic Grandulomatosis With Polyangiitis (EGPA) Study: BITE Unknown status NCT03010436 Phase 2 Benralizumab
14 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
15 Mepolizumab As a Steroid Sparing Treatment Option in the Churg Strauss Syndrome Completed NCT00527566 Phase 1, Phase 2
16 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
17 An Open Trial of the Efficacy of Glucocorticoids and Methotrexate (MTX) in the Treatment of Systemic Vasculitis Completed NCT00001256 Phase 2 prednisone and methotrexate
18 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
19 A Pilot Study on the Use of Rituximab in the Treatment of Churg- Strauss Syndrome With Renal Involvement Terminated NCT00424749 Phase 2 Rituximab;Prednisone
20 A Pilot Study Examining the Effect of Abatacept in ANCA Associated Vasculitis Terminated NCT00482066 Phase 2 Abatacept (Orencia)
21 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
22 Eosinophilic Granulomatosis With Polyangiitis Cohort Unknown status NCT03036670
23 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055 anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone
24 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
25 ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis Unknown status NCT01066208
26 Neutrophils Function and Identification of Prognostic Factors in Granulomatosis With Polyangiitis (Formerly Named Wegener's Granulomatosis). Unknown status NCT01862068
27 VCRC Validation of Patient-Reported Diagnostic Data Completed NCT02190942
28 The ANCA Vasculitis Questionnaire (AAV-PRO©) Completed NCT02507024
29 Infliximab Versus Rituximab in Systemic Necrotizing Vasculitides With Positive ANCA After Relapse or Resistant Immunosuppressant Therapies Completed NCT00307593 Infliximab;Rituximab
30 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
31 Educational Needs of Patients With Systemic Vasculitis- an International Survey Completed NCT02190929
32 Illness Perception, Fatigue, and Function in Systemic Vasculitis (The VCRC Vasculitis Perception (VIP) Study) Completed NCT02190916
33 The Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
34 The Serum Eosinophil Cationic Protein Levels in Behçet's Disease and Its Relation to the Clinical Activity Completed NCT01584778
35 Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Recruiting NCT00315380
36 VCRC Genetic Repository One-Time DNA Protocol Recruiting NCT01241305
37 Clinical and Echocardiographic Evaluation of Patients With Granulomatosis With Polyangiitis Recruiting NCT03782870
38 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
39 VCRC Tissue Biorepository Collection Protocol Recruiting NCT02967068
40 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Recruiting NCT02006134
41 Studies of the Natural History, Pathogenesis, and Outcome of Idiopathic Systemic Vasculitis Recruiting NCT02257866
42 The Vasculitis Pregnancy Registry (V-PREG) Recruiting NCT02593565
43 Single-centre Study Exploring the Utility of Hyperpolarized 129Xe Magnetic Resonance Imaging in Healthy Volunteers and Patients With Lung Disease Recruiting NCT03455686
44 Soluble CD95 Ligand Role in the Pathophysiology of ANCA Associated Vasculitis Recruiting NCT03698071
45 NUCALA ® Subcutaneous Injection Special Drug Use Investigation (EGPA, Long-term) Active, not recruiting NCT03557060 Nucala
46 Defining Immune Tolerance in ANCA-associated Vasculitis (AAV) Terminated NCT01934504
47 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 Churg-Strauss Syndrome

Cochrane evidence based reviews: churg-strauss syndrome

Genetic Tests for Churg-Strauss Syndrome

Anatomical Context for Churg-Strauss Syndrome

MalaCards organs/tissues related to Churg-Strauss Syndrome:

40
Heart, Lung, Skin, Neutrophil, T Cells, Kidney, Testes

Publications for Churg-Strauss Syndrome

Articles related to Churg-Strauss Syndrome:

(show top 50) (show all 2419)
# Title Authors PMID Year
1
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. 61 52
28514601 2017
2
IL-5 pathway inhibition in the treatment of asthma and Churg-Strauss syndrome. 54 61
20513522 2010
3
A comparative study of the diagnostic accuracy of ELISA systems for the detection of anti-neutrophil cytoplasm antibodies available in Japan and Europe. 54 61
19210866 2008
4
Pathways to ANCA production: from differentiation of dendritic cells by proteinase 3 to B lymphocyte maturation in Wegener's granuloma. 54 61
18181035 2008
5
Antibodies to selected minor target antigens in patients with anti-neutrophil cytoplasmic antibodies (ANCA). 54 61
17614969 2007
6
Meta-analysis of myeloperoxidase G-463/A polymorphism in anti-neutrophil cytoplasmic autoantibody-positive vasculitis. 54 61
17521322 2007
7
[Classification of systemic vasculatides]. 54 61
17408915 2007
8
Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. 54 61
17143091 2007
9
TNF-alpha bioactivity-inhibiting therapy in ANCA-associated vasculitis: clinical and experimental considerations. 54 61
17699331 2006
10
Prevalence of antineutrophil cytoplasmic antibodies in patients with various pulmonary diseases or multiorgan dysfunction. 54 61
12687504 2003
11
Autoantibodies in vasculitis. 54 61
12723981 2003
12
Benefit-risk assessment of antileukotrienes in the management of asthma. 54 61
12735786 2003
13
[Microscopic polyangiitis]. 54 61
10896971 2000
14
Distinct differences in autoantigen specificity of anti-neutrophil cytoplasm antibodies in systemic vasculitides and other inflammatory diseases. 54 61
11370121 1999
15
[Leprous neuropathy--observation from the standpoint vasculitis]. 54 61
10481446 1999
16
Pulmonary eosinophilia associated with montelukast. 54 61
10335014 1999
17
What is new with anti-neutrophil cytoplasmic antibodies: diagnostic, pathogenetic and therapeutic implications. 54 61
10456261 1999
18
[Clinical study on five myeloperoxidase specific anti-neutrophil cytoplasmic antibody (MPO-ANCA) positive Churg-Strauss syndrome cases]. 54 61
9852746 1998
19
Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. 54 61
9506572 1998
20
[Eosinophil cationic protein in a 39-year-old patient with Churg-Strauss syndrome]. 54 61
9465849 1998
21
Systemic necrotizing vasculitis. 54 61
9220078 1997
22
[A case of Churg-Strauss syndrome in which MPO-ANCA (antibodies to myeloperoxidase) appeared to reflect the disease activity]. 54 61
7609341 1995
23
[Autoantibodies associated with vasculitis]. 54 61
7933581 1994
24
Correlation of antineutrophil cytoplasmic antibodies with the extrarenal histopathology of Wegener's (pathergic) granulomatosis and related forms of vasculitis. 54 61
8381764 1993
25
Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. 54 61
1466365 1992
26
[Antineutrophil cytoplasmic antibodies--ANCA]. 54 61
1464072 1992
27
Detection of autoantibodies against myeloid lysosomal enzymes: a useful adjunct to classification of patients with biopsy-proven necrotizing arteritis. 54 61
1677531 1991
28
Review of montelukast use over the past 20 years and monitoring of its effects. 61
32023405 2020
29
Pharmacogenetic investigation of efficacy response to mepolizumab in eosinophilic granulomatosis with polyangiitis. 61
32009195 2020
30
Reply to Dr Khan. 61
32012338 2020
31
Biologics and Bronchial Thermoplasty for severe refractory asthma treatment: From eligibility criteria to real practice. A cross-sectional study. 61
31857207 2020
32
Eosinophilic Vasculitis. 61
31927633 2020
33
Targeted Anti-IL-5 Therapies and Future Therapeutics for Hypereosinophilic Syndrome and Rare Eosinophilic Conditions. 61
31919743 2020
34
Anti-interleukin (IL)-5 as a steroid-sparing agent in chronic eosinophilic pneumonia. 61
30444149 2020
35
Treatment and its side effects in ANCA-associated vasculitides - Study based on POLVAS registry data. 61
31958704 2020
36
Predictors of fatal and non-fatal cardiovascular events in ANCA-associated vasculitis: data from the Toronto CanVasc cohort. 61
31958575 2020
37
ANCA-Associated Vasculitis: Core Curriculum 2020. 61
31358311 2020
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Oral corticosteroid-sparing effects of reslizumab in the treatment of eosinophilic granulomatosis with polyangiitis. 61
31984211 2020
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An alternative approach against eosinophils for the treatment of eosinophilic granulomatosis with polyangiitis. 61
32006724 2020
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Large left ventricular non-infectious vegetation in patient with eosinophilic granulomatosis with polyangiitis. 61
31995113 2020
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Eosinophilic Granulomatosis with Polyangiitis: clinical predictors of long-term asthma severity. 61
31958440 2020
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Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease. 61
31762336 2020
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Eosinophilic cholangitis with eosinophilic granulomatosis with polyangiitis: A case report and review of the literature. 61
31481316 2020
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[SYSTEMIC REVIEW OF EOSINOPHILIC DERMATOSES PATIENTS TREATED WITH TNF-Α INHIBITORS AND USTEKINUMAB]. 61
31930806 2020
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Recurrent bilateral eyelid and conjunctival granulomatosis in Churg-Strauss syndrome. 61
32008029 2020
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Systemic hypereosinophilic syndromes: when autoimmunity is Th2 mediated. 61
31985544 2020
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Asthma control in eosinophilic granulomatosis with polyangiitis treated with rituximab. 61
31897956 2020
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"Idiopathic Eosinophilic Vasculitis": Another Side of Hypereosinophilic Syndrome? A Comprehensive Analysis of 117 Cases in Asthma-Free Patients. 61
31863912 2019
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Familial Eosinophilic Granulomatosis with Polyangiitis in a Sister and Brother. 61
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Non-severe eosinophilic granulomatosis with polyangiitis: long-term outcomes after remission-induction trial. 61
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Variations for Churg-Strauss Syndrome

Expression for Churg-Strauss Syndrome

Search GEO for disease gene expression data for Churg-Strauss Syndrome.

Pathways for Churg-Strauss Syndrome

Pathways related to Churg-Strauss Syndrome according to GeneCards Suite gene sharing:

(show all 40)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.98 TNF RNASE3 PRTN3 PRG2 MPO IL5
2
Show member pathways
13.82 TNF PRG2 IL5 IL3 IL2 IL17A
3
Show member pathways
13.68 TNF IL5 IL3 IL2 IL17A IL10
4
Show member pathways
13.55 TNF IL5 IL3 IL2 IL17A IL10
5
Show member pathways
13.38 TNF IL5 IL3 IL2 IL17A IL10
6
Show member pathways
13.37 TNF PRTN3 IL5 IL3 IL2 IL17A
7
Show member pathways
13.21 TNF IL5 IL3 IL2 IL17A IL10
8
Show member pathways
12.64 TNF IL5 IL2 IL17A IL10 CD40LG
9
Show member pathways
12.59 TNF IL5 IL2 IL17A IL10
10
Show member pathways
12.59 TNF MPO IL2 IL17A IL10
11 12.58 TNF MPO IL3 IL2 IL17A IL10
12
Show member pathways
12.44 TNF RNASE3 PRG2 IL5 IL3 IL2
13
Show member pathways
12.38 TNF IL5 IL17A CXCL8 CCL17 CCL11
14
Show member pathways
12.34 IL5 IL3 IL2 IL10
15
Show member pathways
12.31 TNF IL2 IL10 CXCL8 CCL26 CCL17
16
Show member pathways
12.29 TNF IL5 IL3 IL2 IL17A IL10
17
Show member pathways
12.21 TNF IL2 IL10 CCL17
18
Show member pathways
12.07 TNF IL2 IL17A IL10
19 12.04 TNF IL17A IL10 CXCL8 CCL11
20 12.02 TNF IL2 IL10 CXCL8
21 12.02 TNF MPO IL5 IL3 IL2 IL10
22 11.85 TNF IL5 IL2 IL17A IL10
23 11.83 TNF IL17A CXCL8
24 11.8 TNF THBD IL2 IL10
25 11.77 IL5 IL2 CXCL8
26 11.73 TNF IL10 CXCL8
27
Show member pathways
11.71 TNF IL2 CXCL8 CD40LG
28 11.71 TNF IL5 CXCL8 CCL11
29
Show member pathways
11.68 TNF IL5 IL3 IL2 CXCL8 CD40LG
30 11.59 TNF IL10 CXCL8 CD40LG
31 11.55 TNF IL2 IL17A IL10 CCL26 CCL17
32 11.53 TNF IL10 CXCL8
33 11.53 IL5 IL10 CD40LG CCL26 CCL17 CCL11
34 11.52 TNF MPO HMGB1
35 11.52 IL5 IL2 IL10 CCL17 CCL11
36 11.34 IL5 IL2 CD40LG
37 11.32 TNF IL5 IL17A
38 11.23 TNF IL5 IL3 IL2 IL10
39 11.22 IL5 IL2 IL10
40 10.3 TNF PRG2 IL5 IL3 IL2 IL17A

GO Terms for Churg-Strauss Syndrome

Cellular components related to Churg-Strauss Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.89 TNF RNASE3 PRTN3 PRG2 MPO IL5
2 extracellular space GO:0005615 9.53 TNF THBD RNASE3 PRTN3 MPO IL5
3 azurophil granule lumen GO:0035578 9.33 RNASE3 PRTN3 MPO

Biological processes related to Churg-Strauss Syndrome according to GeneCards Suite gene sharing:

(show all 29)
# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.95 RNASE3 PRTN3 PRG2 MPO HMGB1 COTL1
2 MAPK cascade GO:0000165 9.93 TNF IL5 IL3 IL2
3 cellular response to lipopolysaccharide GO:0071222 9.9 TNF IL10 HMGB1 CXCL8
4 defense response to bacterium GO:0042742 9.89 TNF RNASE3 PRG2 MPO IL10
5 defense response GO:0006952 9.88 TNF MPO CYSLTR1 CXCL8
6 cellular response to tumor necrosis factor GO:0071356 9.87 CXCL8 CCL26 CCL17 CCL11
7 cell-cell signaling GO:0007267 9.85 IL3 IL2 IL17A CCL26 CCL17
8 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.83 TNF HMGB1 CCL26 CCL17 CCL11
9 cytokine-mediated signaling pathway GO:0019221 9.81 TNF PRTN3 IL5 IL3 IL2 IL17A
10 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.8 TNF IL3 IL2
11 positive regulation of endothelial cell proliferation GO:0001938 9.79 IL10 CCL26 CCL11
12 monocyte chemotaxis GO:0002548 9.75 CCL26 CCL17 CCL11
13 lymphocyte chemotaxis GO:0048247 9.73 CCL26 CCL17 CCL11
14 chemotaxis GO:0006935 9.73 HMGB1 CYSLTR1 CXCL8 CCL26 CCL17 CCL11
15 cellular response to interleukin-1 GO:0071347 9.72 IL17A CXCL8 CCL26 CCL17 CCL11
16 positive regulation of interleukin-6 secretion GO:2000778 9.71 TNF IL17A HMGB1
17 neutrophil chemotaxis GO:0030593 9.71 CXCL8 CCL26 CCL17 CCL11
18 defense response to fungus GO:0050832 9.7 MPO IL17A COTL1
19 positive regulation of JAK-STAT cascade GO:0046427 9.69 TNF IL5 IL10
20 chemokine-mediated signaling pathway GO:0070098 9.67 CXCL8 CCL26 CCL17 CCL11
21 inflammatory response GO:0006954 9.65 TNF IL5 IL17A IL10 HMGB1 CXCL8
22 positive regulation of podosome assembly GO:0071803 9.64 TNF IL5
23 positive regulation of immunoglobulin secretion GO:0051024 9.64 IL5 IL2
24 response to molecule of bacterial origin GO:0002237 9.63 IL10 CXCL8
25 endothelial cell apoptotic process GO:0072577 9.61 TNF IL10
26 negative regulation of cytokine secretion involved in immune response GO:0002740 9.61 TNF IL10
27 regulation of immunoglobulin secretion GO:0051023 9.59 TNF CD40LG
28 receptor biosynthetic process GO:0032800 9.55 TNF IL10
29 immune response GO:0006955 9.4 TNF PRG2 IL5 IL3 IL2 IL17A

Molecular functions related to Churg-Strauss Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 growth factor activity GO:0008083 9.62 IL5 IL3 IL2 IL10
2 cytokine activity GO:0005125 9.4 TNF IL5 IL3 IL2 IL17A IL10
3 CCR chemokine receptor binding GO:0048020 9.33 CCL26 CCL17 CCL11
4 CCR3 chemokine receptor binding GO:0031728 9.26 CCL26 CCL11
5 chemokine activity GO:0008009 9.26 CXCL8 CCL26 CCL17 CCL11

Sources for Churg-Strauss Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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