CSS
MCID: CHR001
MIFTS: 61

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 polyarteritis nodosa, 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 lung, heart 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), formerly 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 846)
# Related Disease Score Top Affiliating Genes
1 microscopic polyangiitis 33.5 TNF MPO HLA-DRB1
2 polyarteritis nodosa 31.9 PRTN3 MPO HLA-DRB1
3 granulomatous angiitis 31.9 PRTN3 IL5
4 mononeuritis multiplex 31.8 TNF PRTN3 MPO CD40LG
5 autoimmune lymphoproliferative syndrome 31.7 TNF IL2 IL10 CD40LG
6 anca-associated vasculitis 31.6 PRTN3 MPO
7 rapidly progressive glomerulonephritis 31.5 PRTN3 MPO CD40LG
8 vasculitis 31.5 TNF THBD PRTN3 MPO IL17A IL10
9 chronic eosinophilic pneumonia 31.5 RNASE3 IL5 CCL11
10 purpura 31.5 TNF THBD IL10 CD40LG
11 mononeuropathy 31.4 TNF PRTN3 MPO CD40LG
12 glomerulonephritis 31.4 PRTN3 MPO IL1B CD40LG
13 ige responsiveness, atopic 31.3 RNASE3 IL5 IL10
14 chronic rhinitis 31.2 RNASE3 MPO IL5
15 maxillary sinusitis 31.2 RNASE3 PRTN3 MPO CD40LG
16 eosinophilic gastritis 30.9 IL5 CCL26 CCL11
17 guillain-barre syndrome 30.9 TNF HLA-DRB1 CD40LG
18 scleritis 30.9 TNF PRTN3 IL5
19 allergic bronchopulmonary aspergillosis 30.9 IL5 IL10 HLA-DRB1 CCL17
20 granulomatosis with polyangiitis 30.9 TNF PRTN3 MPO IL5 IL17A CD40LG
21 acute myocarditis 30.8 TNF IL1B IL10
22 multifocal choroiditis 30.8 TNF IL10
23 mastoiditis 30.7 PRTN3 MPO CD40LG
24 eosinophilic pneumonia 30.7 RNASE3 IL5 IL3 CXCL8 CCL17 CCL11
25 pericarditis 30.7 TNF IL1B CXCL8 CD40LG
26 proctitis 30.6 TNF RNASE3 MPO
27 polyradiculoneuropathy 30.6 IL17A IL10 HLA-DRB1
28 endomyocardial fibrosis 30.6 TNF RNASE3 PRG2 IL5 IL10 CCL26
29 henoch-schoenlein purpura 30.6 RNASE3 PRTN3 MPO CD40LG
30 optic nerve disease 30.6 TNF IL1B IL17A CD40LG
31 neuritis 30.5 TNF IL1B IL10 CXCL8
32 endocarditis 30.5 TNF IL17A IL10 CXCL8
33 goodpasture syndrome 30.5 PRTN3 MPO HLA-DRB1 CD40LG
34 cryptococcosis 30.5 TNF IL17A IL10
35 hypersensitivity vasculitis 30.4 TNF PRTN3 MPO CD40LG
36 severe cutaneous adverse reaction 30.4 TNF HLA-DRB1 CD40LG
37 autoimmune hepatitis 30.4 TNF IL10 HLA-DRB1 CD40LG
38 eosinophilic gastroenteritis 30.4 RNASE3 PRG2 IL5 IL3 CCL26 CCL11
39 recurrent respiratory papillomatosis 30.4 IL2 HLA-DRB1
40 allergic rhinitis 30.3 RNASE3 MPO IL5 IL17A IL10 CXCL8
41 gastric ulcer 30.3 TNF IL1B CXCL8
42 stevens-johnson syndrome/toxic epidermal necrolysis 30.3 IL5 IL2 CCL17
43 thyroiditis 30.3 TNF IL2 IL10
44 spondylitis 30.3 TNF IL17A IL10
45 macular retinal edema 30.3 TNF IL1B CXCL8
46 loeffler endocarditis 30.2 RNASE3 PRG2 IL5 IL3
47 spondyloarthropathy 1 30.2 TNF PRTN3 IL17A CD40LG
48 prostatitis 30.2 TNF IL1B IL10 CXCL8
49 iridocyclitis 30.2 TNF IL17A IL10 CD40LG
50 choroiditis 30.2 TNF IL17A IL10 CD40LG

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 54)
# 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 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
14 gait disturbance 58 31 frequent (33%) Frequent (79-30%) HP:0001288
15 hypertrophic cardiomyopathy 58 31 frequent (33%) Frequent (79-30%) HP:0001639
16 dysphagia 58 31 frequent (33%) Frequent (79-30%) HP:0002015
17 skin rash 58 31 frequent (33%) Frequent (79-30%) HP:0000988
18 arthralgia 58 31 frequent (33%) Frequent (79-30%) HP:0002829
19 hematuria 58 31 frequent (33%) Frequent (79-30%) HP:0000790
20 hypopigmented skin patches 58 31 frequent (33%) Frequent (79-30%) HP:0001053
21 abnormal pericardium morphology 58 31 frequent (33%) Frequent (79-30%) HP:0001697
22 nausea and vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002017
23 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
24 hypertension 58 31 frequent (33%) Frequent (79-30%) HP:0000822
25 venous thrombosis 58 31 frequent (33%) Frequent (79-30%) HP:0004936
26 tubulointerstitial nephritis 58 31 frequent (33%) Frequent (79-30%) HP:0001970
27 abnormal pleura morphology 31 frequent (33%) HP:0002103
28 arthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001369
29 malabsorption 58 31 occasional (7.5%) Occasional (29-5%) HP:0002024
30 gastroesophageal reflux 58 31 occasional (7.5%) Occasional (29-5%) HP:0002020
31 proteinuria 58 31 occasional (7.5%) Occasional (29-5%) HP:0000093
32 renal insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0000083
33 fever 58 31 occasional (7.5%) Occasional (29-5%) HP:0001945
34 cranial nerve paralysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0006824
35 acrocyanosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001063
36 hemiplegia/hemiparesis 58 31 occasional (7.5%) Occasional (29-5%) HP:0004374
37 subcutaneous nodule 58 31 occasional (7.5%) Occasional (29-5%) HP:0001482
38 papule 58 31 occasional (7.5%) Occasional (29-5%) HP:0200034
39 myocardial infarction 58 31 occasional (7.5%) Occasional (29-5%) HP:0001658
40 myalgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003326
41 cough 58 31 occasional (7.5%) Occasional (29-5%) HP:0012735
42 myositis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100614
43 respiratory insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0002093
44 transient ischemic attack 58 31 occasional (7.5%) Occasional (29-5%) HP:0002326
45 glomerulopathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0100820
46 hemoptysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002105
47 myocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012819
48 endocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100584
49 recurrent intrapulmonary hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0006535
50 intestinal obstruction 58 31 occasional (7.5%) Occasional (29-5%) HP:0005214

Symptoms:

12
  • asthma

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

26 (show all 40)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 10.27 CYSLTR1 IL10 IL1B
2 Increased shRNA abundance (Z-score > 2) GR00366-A-102 10.27 IL1B
3 Increased shRNA abundance (Z-score > 2) GR00366-A-112 10.27 COTL1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-113 10.27 IL10
5 Increased shRNA abundance (Z-score > 2) GR00366-A-12 10.27 CYSLTR1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-125 10.27 IL10
7 Increased shRNA abundance (Z-score > 2) GR00366-A-126 10.27 MPO
8 Increased shRNA abundance (Z-score > 2) GR00366-A-14 10.27 IL1B
9 Increased shRNA abundance (Z-score > 2) GR00366-A-152 10.27 IL10
10 Increased shRNA abundance (Z-score > 2) GR00366-A-157 10.27 MPO
11 Increased shRNA abundance (Z-score > 2) GR00366-A-161 10.27 IL10
12 Increased shRNA abundance (Z-score > 2) GR00366-A-170 10.27 IL1B
13 Increased shRNA abundance (Z-score > 2) GR00366-A-181 10.27 IL1B
14 Increased shRNA abundance (Z-score > 2) GR00366-A-184 10.27 MPO
15 Increased shRNA abundance (Z-score > 2) GR00366-A-198 10.27 IL10
16 Increased shRNA abundance (Z-score > 2) GR00366-A-199 10.27 IL1B
17 Increased shRNA abundance (Z-score > 2) GR00366-A-202 10.27 COTL1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-210 10.27 IL10
19 Increased shRNA abundance (Z-score > 2) GR00366-A-214 10.27 IL10
20 Increased shRNA abundance (Z-score > 2) GR00366-A-29 10.27 MPO
21 Increased shRNA abundance (Z-score > 2) GR00366-A-4 10.27 CYSLTR1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-40 10.27 MPO
23 Increased shRNA abundance (Z-score > 2) GR00366-A-50 10.27 IL1B
24 Increased shRNA abundance (Z-score > 2) GR00366-A-51 10.27 IL1B
25 Increased shRNA abundance (Z-score > 2) GR00366-A-52 10.27 IL10
26 Increased shRNA abundance (Z-score > 2) GR00366-A-53 10.27 IL10
27 Increased shRNA abundance (Z-score > 2) GR00366-A-57 10.27 MPO
28 Increased shRNA abundance (Z-score > 2) GR00366-A-62 10.27 CYSLTR1
29 Increased shRNA abundance (Z-score > 2) GR00366-A-65 10.27 IL10
30 Increased shRNA abundance (Z-score > 2) GR00366-A-67 10.27 IL10
31 Increased shRNA abundance (Z-score > 2) GR00366-A-68 10.27 IL10
32 Increased shRNA abundance (Z-score > 2) GR00366-A-7 10.27 MPO
33 Increased shRNA abundance (Z-score > 2) GR00366-A-70 10.27 MPO
34 Increased shRNA abundance (Z-score > 2) GR00366-A-79 10.27 IL1B
35 Increased shRNA abundance (Z-score > 2) GR00366-A-81 10.27 CYSLTR1 IL1B
36 Increased shRNA abundance (Z-score > 2) GR00366-A-82 10.27 IL10
37 Increased shRNA abundance (Z-score > 2) GR00366-A-85 10.27 IL10
38 Increased shRNA abundance (Z-score > 2) GR00366-A-98 10.27 COTL1
39 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.47 CD40LG CXCL8 IL10 IL17A IL1B MPO
40 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.47 CXCL8 IL10 IL17A IL1B IL2 MPO

MGI Mouse Phenotypes related to Churg-Strauss Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 CCL11 CCL17 CD40LG IL10 IL17A IL1B
2 immune system MP:0005387 9.8 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 85)
# 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
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
5
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
6 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
7
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
8
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
9
leucovorin Approved Phase 4 58-05-9 6006 143
10
Methotrexate Approved Phase 4 59-05-2, 1959-05-2 126941
11
Mycophenolic acid Approved Phase 4 24280-93-1 446541
12
Hydroxychloroquine Approved Phase 4 118-42-3 3652
13
Povidone Approved Phase 4 9003-39-8
14
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
15
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
16 Alkylating Agents Phase 4
17 Hormones Phase 4
18 Antineoplastic Agents, Hormonal Phase 4
19 Methylprednisolone Acetate Phase 4
20 glucocorticoids Phase 4
21 Hormone Antagonists Phase 4
22 Anti-Inflammatory Agents Phase 4
23 Gastrointestinal Agents Phase 4
24 Dermatologic Agents Phase 4
25 Folic Acid Antagonists Phase 4
26 Vitamin B Complex Phase 4
27 Folate Phase 4
28 Vitamin B9 Phase 4
29 Antiemetics Phase 4
30 Neuroprotective Agents Phase 4
31 Protective Agents Phase 4
32 Antitubercular Agents Phase 4
33 Anti-Bacterial Agents Phase 4
34 Antibiotics, Antitubercular Phase 4
35 Antimetabolites Phase 4
36 Anti-Infective Agents Phase 4
37 Antiparasitic Agents Phase 4
38 Antiprotozoal Agents Phase 4
39 Antimalarials Phase 4
40 Benralizumab Approved, Investigational Phase 3 1044511-01-4
41 Respiratory System Agents Phase 3
42 Pharmaceutical Solutions Phase 3
43 Anti-Asthmatic Agents Phase 3
44 Immunoglobulins Phase 3
45 Antibodies Phase 3
46 Immunoglobulins, Intravenous Phase 3
47 Rho(D) Immune Globulin Phase 3
48 gamma-Globulins Phase 3
49 Immunoglobulin G Phase 3
50
Reslizumab Approved, Investigational Phase 2 241473-69-8

Interventional clinical trials:

(show top 50) (show all 51)
# 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 Hydroxychloroquine in ANCA Vasculitis Evaluation - A Multicentre, Randomised, Double-blind, Placebo-controlled Trial Not yet recruiting NCT04316494 Phase 4 Hydroxychloroquine;Placebo
5 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
6 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
7 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
8 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
9 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
10 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
11 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)
12 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
13 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
14 The Efficacy and Safety of Benralizumab In the Treatment of Eosinophilic Grandulomatosis With Polyangiitis (EGPA) Study: BITE Unknown status NCT03010436 Phase 2 Benralizumab
15 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
16 Mepolizumab As a Steroid Sparing Treatment Option in the Churg Strauss Syndrome Completed NCT00527566 Phase 1, Phase 2
17 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
18 An Open Trial of the Efficacy of Glucocorticoids and Methotrexate (MTX) in the Treatment of Systemic Vasculitis Completed NCT00001256 Phase 2 prednisone and methotrexate
19 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
20 A Pilot Study on the Use of Rituximab in the Treatment of Churg- Strauss Syndrome With Renal Involvement Terminated NCT00424749 Phase 2 Rituximab;Prednisone
21 A Pilot Study Examining the Effect of Abatacept in ANCA Associated Vasculitis Terminated NCT00482066 Phase 2 Abatacept (Orencia)
22 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
23 Eosinophilic Granulomatosis With Polyangiitis Cohort Unknown status NCT03036670
24 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Unknown status NCT02006134
25 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055 anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone
26 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
27 ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis Unknown status NCT01066208
28 Neutrophils Function and Identification of Prognostic Factors in Granulomatosis With Polyangiitis (Formerly Named Wegener's Granulomatosis). Unknown status NCT01862068
29 VCRC Validation of Patient-Reported Diagnostic Data Completed NCT02190942
30 The ANCA Vasculitis Questionnaire (AAV-PRO©) Completed NCT02507024
31 Infliximab Versus Rituximab in Systemic Necrotizing Vasculitides With Positive ANCA After Relapse or Resistant Immunosuppressant Therapies Completed NCT00307593 Infliximab;Rituximab
32 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
33 Educational Needs of Patients With Systemic Vasculitis- an International Survey Completed NCT02190929
34 Illness Perception, Fatigue, and Function in Systemic Vasculitis (The VCRC Vasculitis Perception (VIP) Study) Completed NCT02190916
35 The Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
36 The Serum Eosinophil Cationic Protein Levels in Behçet's Disease and Its Relation to the Clinical Activity Completed NCT01584778
37 Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Recruiting NCT00315380
38 Clinical and Echocardiographic Evaluation of Patients With Granulomatosis With Polyangiitis Recruiting NCT03782870
39 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
40 VCRC Tissue Biorepository Collection Protocol Recruiting NCT02967068
41 Studies of the Natural History, Pathogenesis, and Outcome of Idiopathic Systemic Vasculitis Recruiting NCT02257866
42 Urinary CD4+ Effector Memory T Cells as Biomarkers for Prediction of Outcome in ANCA Glomerulonephritis Recruiting NCT04320667
43 The Vasculitis Pregnancy Registry (V-PREG) Recruiting NCT02593565
44 Single-centre Study Exploring the Utility of Hyperpolarized 129Xe Magnetic Resonance Imaging in Healthy Volunteers and Patients With Lung Disease Recruiting NCT03455686
45 Soluble CD95 Ligand Role in the Pathophysiology of ANCA Associated Vasculitis Recruiting NCT03698071
46 Urinary T Lymphocytes Predict Renal Flares in Patients With Inactive Anti-neutrophil Cytoplasmic Antibody (ANCA) Associated Glomerulonephritis Recruiting NCT04428398
47 NUCALA ® Subcutaneous Injection Special Drug Use Investigation (EGPA, Long-term) Active, not recruiting NCT03557060 Nucala
48 VCRC Genetic Repository One-Time DNA Protocol Active, not recruiting NCT01241305
49 A Local Interventional Study in a Cohort of Patients With GPA, MPA and EGPA (PART 2 of "Vitamin D Status in ANCA-associated Vasculitis: Analysis of RDCRN-VCRC and Local Clinic Cohorts of Patients With GPA, MPA and EGPA") Not yet recruiting NCT04280601
50 Defining Immune Tolerance in ANCA-associated Vasculitis (AAV) Terminated NCT01934504

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

Publications for Churg-Strauss Syndrome

Articles related to Churg-Strauss Syndrome:

(show top 50) (show all 2467)
# Title Authors PMID Year
1
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. 52 61
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. 61 54
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. 61 54
17521322 2007
7
[Classification of systemic vasculatides]. 61 54
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. 61 54
17699331 2006
10
Prevalence of antineutrophil cytoplasmic antibodies in patients with various pulmonary diseases or multiorgan dysfunction. 61 54
12687504 2003
11
Benefit-risk assessment of antileukotrienes in the management of asthma. 61 54
12735786 2003
12
Autoantibodies in vasculitis. 61 54
12723981 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. 61 54
11370121 1999
15
[Leprous neuropathy--observation from the standpoint vasculitis]. 61 54
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]. 61 54
9465849 1998
21
Systemic necrotizing vasculitis. 61 54
9220078 1997
22
[A case of Churg-Strauss syndrome in which MPO-ANCA (antibodies to myeloperoxidase) appeared to reflect the disease activity]. 61 54
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. 61 54
8381764 1993
25
Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. 61 54
1466365 1992
26
[Antineutrophil cytoplasmic antibodies--ANCA]. 61 54
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
Eosinophilic granulomatosis with polyangiitis presenting with myositis: case based review. 61
32270295 2020
29
Pulmonary Eosinophilic Granulomatosis with Polyangiitis Has IgG4 Plasma Cells and Immunoregulatory Features. 61
32251643 2020
30
Multiple ulcerations and perforation in the small intestine after steroid treatment in eosinophilic granulomatosis with polyangiitis: a case report and literature review. 61
32151788 2020
31
Clinical utility of serum IgG4 measurement. 61
32272158 2020
32
Retinal artery occlusion followed by contralateral amaurosis fugax in association with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). 61
32309675 2020
33
Demographic and clinical characteristics of patients with ANCA-positive vasculitis in a Colombian University Hospital over a 12-year period: 2005-2017. 61
32564160 2020
34
Reduction of Myeloperoxidase-Antineutrophil Cytoplasmic Antibody by Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis. 61
32568949 2020
35
Eosinophilic Granulomatosis With Polyangiitis: An Unusual Case of Pediatric Subglottic Stenosis. 61
32562500 2020
36
Eosinophilic granulomatosis with polyangiitis associated myocarditis: role of myocardial tissue characterization. 61
31848591 2020
37
An alternative approach against eosinophils for the treatment of eosinophilic granulomatosis with polyangiitis. 61
32006724 2020
38
Lung-limited or lung-dominant variant of eosinophilic granulomatosis with polyangiitis. 61
32061719 2020
39
Benralizumab as a glucocorticoid-sparing treatment option for severe asthma in eosinophilic granulomatosis with polyangiitis. 61
32505716 2020
40
An increased disease burden of autoimmune inflammatory rheumatic diseases in Korea. 61
31852583 2020
41
Eosinophilic granulomatosis with polyangiitis mimicking COVID-19: a case report. 61
32495957 2020
42
FCGR3B polymorphism predicts relapse risk in eosinophilic granulomatosis with polyangiitis. 61
32375167 2020
43
Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management. 61
32348510 2020
44
Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Benralizumab. 61
32407950 2020
45
[A perimyocarditis revealing eosinophilic granulomatosis with polyangiitis]. 61
32265026 2020
46
Eosinophilic Dermatoses: Recognition and Management. 61
32394361 2020
47
Asthma control in eosinophilic granulomatosis with polyangiitis treated with rituximab. 61
31897956 2020
48
Testicular infarction and Pulmonary embolism secondary to Non-asthmatic Eosinophilic Granulomatosis with Polyangiitis: a case report. 61
32376517 2020
49
Eosinophilic Granulomatosis With Polyangiitis: Clinical Predictors of Long-term Asthma Severity. 61
31958440 2020
50
Benralizumab as a Potential Adjunctive Therapy in Eosinophilic Granulomatosis With Polyangiitis. 61
32453217 2020

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 top 50) (show all 57)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
14.01 TNF RNASE3 PRTN3 PRG2 MPO IL5
2
Show member pathways
13.88 TNF PRG2 IL5 IL3 IL2 IL1B
3
Show member pathways
13.74 TNF IL5 IL3 IL2 IL1B IL17A
4
Show member pathways
13.62 TNF IL5 IL3 IL2 IL1B IL17A
5
Show member pathways
13.38 TNF IL5 IL3 IL2 IL1B IL17A
6
Show member pathways
13.26 TNF PRTN3 IL5 IL3 IL2 IL1B
7
Show member pathways
13.1 TNF IL5 IL3 IL2 IL1B IL17A
8
Show member pathways
12.82 TNF IL2 IL1B HLA-DRB1 CXCL8
9
Show member pathways
12.73 TNF IL3 IL2 IL1B CD40LG
10
Show member pathways
12.7 TNF IL5 IL2 IL1B IL17A IL10
11
Show member pathways
12.69 TNF IL5 IL2 IL1B IL17A IL10
12
Show member pathways
12.67 TNF IL2 IL10 HLA-DRB1 CD40LG
13
Show member pathways
12.67 TNF MPO IL2 IL1B IL17A IL10
14 12.59 TNF MPO IL3 IL2 IL1B IL17A
15
Show member pathways
12.41 TNF IL5 IL1B IL17A CXCL8 CCL17
16
Show member pathways
12.4 TNF IL2 IL10 CXCL8 CCL26 CCL17
17
Show member pathways
12.35 TNF MPO IL2 IL1B
18
Show member pathways
12.34 TNF IL1B IL10 HLA-DRB1 CD40LG
19
Show member pathways
12.31 TNF IL5 IL3 IL2 IL1B IL17A
20
Show member pathways
12.3 TNF IL2 IL1B IL10 CCL17
21 12.27 TNF IL1B IL10 HLA-DRB1
22
Show member pathways
12.23 TNF IL1B CXCL8 CCL11
23 12.21 TNF IL1B IL10 CXCL8
24
Show member pathways
12.18 TNF RNASE3 PRG2 IL5 IL3 IL2
25 12.13 TNF IL2 IL1B IL10 CXCL8
26 12.11 TNF MPO IL5 IL3 IL2 IL10
27 12.08 TNF IL1B IL17A IL10 CXCL8 CCL11
28 12.07 TNF IL2 IL1B CXCL8
29
Show member pathways
12.07 TNF IL2 IL1B IL17A IL10
30 12.03 TNF IL5 IL3 IL1B HLA-DRB1
31 12 TNF IL1B CXCL8 CD40LG
32 11.99 TNF IL1B IL10 CXCL8
33 11.99 TNF IL1B IL17A HLA-DRB1 CXCL8
34 11.98 TNF THBD IL1B CXCL8
35 11.87 TNF THBD IL2 IL1B IL10
36 11.85 TNF IL1B IL10 CXCL8
37 11.81 IL5 IL2 CXCL8
38 11.81 TNF IL5 IL2 IL1B IL17A IL10
39
Show member pathways
11.79 TNF IL2 IL1B CXCL8 CD40LG
40 11.78 TNF IL5 IL1B CXCL8 CCL11
41
Show member pathways
11.76 TNF IL5 IL3 IL2 CXCL8 CD40LG
42 11.67 TNF IL1B IL10 CXCL8 CD40LG
43 11.65 TNF IL1B CXCL8
44 11.65 TNF IL1B IL10 CXCL8
45 11.62 IL5 IL2 IL10 CCL17 CCL11
46 11.6 IL5 IL10 CD40LG CCL26 CCL17 CCL11
47 11.56 IL5 IL3 IL1B
48 11.56 TNF IL2 IL1B
49 11.44 TNF IL1B IL10
50 11.44 TNF IL5 IL1B 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 external side of plasma membrane GO:0009897 9.55 TNF THBD IL17A HLA-DRB1 CD40LG
3 extracellular space GO:0005615 9.55 TNF THBD RNASE3 PRTN3 MPO IL5

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

(show all 42)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.3 IL5 IL1B IL10 HLA-DRB1 CYSLTR1 CXCL8
2 neutrophil degranulation GO:0043312 10.08 RNASE3 PRTN3 PRG2 MPO COTL1
3 positive regulation of protein phosphorylation GO:0001934 9.96 TNF IL2 IL1B HLA-DRB1
4 cellular response to lipopolysaccharide GO:0071222 9.96 TNF IL1B IL10 CXCL8
5 response to lipopolysaccharide GO:0032496 9.95 THBD MPO IL1B IL10
6 MAPK cascade GO:0000165 9.93 TNF IL5 IL3 IL2 IL1B
7 defense response GO:0006952 9.92 TNF MPO CYSLTR1 CXCL8
8 defense response to bacterium GO:0042742 9.92 TNF RNASE3 PRG2 MPO IL10
9 cellular response to tumor necrosis factor GO:0071356 9.9 CXCL8 CCL26 CCL17 CCL11
10 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.88 TNF HLA-DRB1 CCL26 CCL17 CCL11
11 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.86 TNF IL3 IL2
12 positive regulation of DNA-binding transcription factor activity GO:0051091 9.86 TNF IL5 IL1B IL10
13 positive regulation of endothelial cell proliferation GO:0001938 9.85 IL10 CCL26 CCL11
14 cell-cell signaling GO:0007267 9.85 IL3 IL2 IL1B IL17A CCL26 CCL17
15 positive regulation of T cell proliferation GO:0042102 9.84 IL2 IL1B CD40LG
16 inflammatory response GO:0006954 9.81 TNF IL5 IL1B IL17A CXCL8 CD40LG
17 positive regulation of interferon-gamma production GO:0032729 9.8 TNF IL2 IL1B
18 monocyte chemotaxis GO:0002548 9.8 CCL26 CCL17 CCL11
19 chemotaxis GO:0006935 9.8 CYSLTR1 CXCL8 CCL26 CCL17 CCL11
20 positive regulation of interleukin-6 secretion GO:2000778 9.79 TNF IL1B IL17A
21 lymphocyte chemotaxis GO:0048247 9.77 CCL26 CCL17 CCL11
22 chemokine-mediated signaling pathway GO:0070098 9.76 CXCL8 CCL26 CCL17 CCL11
23 positive regulation of JAK-STAT cascade GO:0046427 9.74 TNF IL5 IL10
24 defense response to fungus GO:0050832 9.72 MPO IL17A COTL1
25 cellular response to interleukin-1 GO:0071347 9.72 IL17A CXCL8 CCL26 CCL17 CCL11
26 response to molecule of bacterial origin GO:0002237 9.69 IL10 CXCL8
27 negative regulation of neurogenesis GO:0050768 9.69 TNF IL1B CCL11
28 positive regulation of interleukin-1 beta biosynthetic process GO:0050725 9.68 TNF IL17A
29 regulation of establishment of endothelial barrier GO:1903140 9.67 TNF IL1B
30 endothelial cell apoptotic process GO:0072577 9.67 TNF IL10
31 inflammatory response to antigenic stimulus GO:0002437 9.67 IL1B HLA-DRB1 CYSLTR1
32 negative regulation of cytokine production involved in immune response GO:0002719 9.65 TNF IL10
33 neutrophil chemotaxis GO:0030593 9.65 IL1B CXCL8 CCL26 CCL17 CCL11
34 cytokine-mediated signaling pathway GO:0019221 9.65 TNF PRTN3 IL5 IL3 IL2 IL1B
35 regulation of immunoglobulin secretion GO:0051023 9.64 TNF CD40LG
36 positive regulation of fever generation GO:0031622 9.64 TNF IL1B
37 negative regulation of cytokine secretion involved in immune response GO:0002740 9.63 TNF IL10
38 sequestering of triglyceride GO:0030730 9.61 TNF IL1B
39 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.61 TNF IL1B IL10
40 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.58 TNF IL1B
41 receptor biosynthetic process GO:0032800 9.58 TNF IL10
42 immune response GO:0006955 9.47 TNF PRG2 IL5 IL3 IL2 IL1B

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 9.56 IL5 IL3 IL2 IL10
2 cytokine activity GO:0005125 9.4 TNF IL5 IL3 IL2 IL1B IL17A
3 CCR chemokine receptor binding GO:0048020 9.33 CCL26 CCL17 CCL11
4 CCR3 chemokine receptor binding GO:0031728 9.32 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
Content
Loading form....