CSS
MCID: CHR001
MIFTS: 62

Churg-Strauss Syndrome (CSS)

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Immune diseases, Nephrological diseases, Neuronal diseases, Rare diseases, Respiratory diseases
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Aliases & Classifications for Churg-Strauss Syndrome

MalaCards integrated aliases for Churg-Strauss Syndrome:

Name: Churg-Strauss Syndrome 11 19 58 75 53 43 14 16 71 75 33
Eosinophilic Granulomatosis with Polyangiitis 19 58 75 33
Allergic Granulomatous Angiitis 11 75 33
Churg-Strauss Vasculitis 11 19 33
Granulomatous Allergic Angiitis 19 58
Allergic Angiitis 19 75
Egpa 19 58
Allergic Angiitis and Granulomatosis 19
Allergic Granulomatous and Angiitis 19
Allergic Granulomatosis Angiitis 11
Allergic Granulomatosis 19
Css 19

Characteristics:


Prevelance:

Eosinophilic Granulomatosis with Polyangiitis: 1-9/100000 (Europe, France, Sweden, Norway, Australia, Worldwide, Germany, Japan) <1/1000000 (Sweden, United Kingdom, Spain, Norway, Turkey, Peru) 1-9/1000000 (Europe, Germany, Lithuania, France, United States) 58

Age Of Onset:

Eosinophilic Granulomatosis with Polyangiitis: Adolescent,Adult,Elderly 58

Age Of Death:

Eosinophilic Granulomatosis with Polyangiitis: adolescent,adult,elderly,young Adult 58

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

Disease Ontology: 11 A vasculitits that is systemic vasculitis realized as blood vessel inflammation and has symptom asthma along with hay fever, rash and gastrointestinal bleeding.

MalaCards based summary: Churg-Strauss Syndrome, also known as eosinophilic granulomatosis with polyangiitis, is related to polyarteritis nodosa 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 Prednisone and Levoleucovorin have been mentioned in the context of this disorder. Affiliated tissues include lung, bone and neutrophil, and related phenotypes are sinusitis and congestive heart failure

GARD: 19 An autoimmune necrotizing vasculitis with the formation of granulomas. It is a pulmonary and systemic vasculitis associated with eosinophilia.

Orphanet: 58 A rare systemic vasculitis of small vessels characterized by asthma, blood and tissue eosinophilia and vasculitis manifestations.

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

Allergic granulomatous angiitis: Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins... 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 990)
# Related Disease Score Top Affiliating Genes
1 polyarteritis nodosa 31.9 TNF PRTN3 MPO HLA-DRB1
2 mononeuritis multiplex 31.5 TNF PRTN3 MPO IL5 HLA-DRB1
3 hypersensitivity vasculitis 31.4 TNF PRTN3 MPO
4 mononeuropathy 31.4 TNF PRTN3 MPO
5 rapidly progressive glomerulonephritis 31.3 PRTN3 MPO HLA-DRB1
6 chronic eosinophilic pneumonia 31.2 RNASE3 IL5 CCL11
7 microscopic polyangiitis 31.2 PRTN3 MPO IL2RA HLA-DRB1
8 glomerulonephritis 31.2 TNF PRTN3 MPO IL1B IL10
9 ige responsiveness, atopic 31.2 RNASE3 IL5 IL10
10 autoimmune lymphoproliferative syndrome 31.1 TNF IL2RA IL2 IL17A IL10
11 maxillary sinusitis 31.1 RNASE3 PRTN3 MPO
12 crescentic glomerulonephritis 31.0 TNF PRTN3 MPO IL17A
13 pericarditis 30.9 TNF IL1B CXCL8
14 hypereosinophilic syndrome, idiopathic 30.9 IL5 IL3
15 vasculitis 30.8 TNF THBD PRTN3 MPO IL1B IL17A
16 cranial nerve palsy 30.8 TNF PRTN3 MPO
17 eosinophilic gastroenteritis 30.8 RNASE3 IL5 IL3 CCL26 CCL11
18 guillain-barre syndrome 30.8 TNF IL17A HLA-DRB1
19 endomyocardial fibrosis 30.7 TNF RNASE3 PRG2 IL5 IL10 CCL26
20 allergic rhinitis 30.7 RNASE3 MPO IL5 IL17A IL10 CXCL8
21 acute myocarditis 30.7 TNF IL1B IL10
22 optic nerve disease 30.7 TNF IL1B IL17A IL10 HLA-DRB1
23 eosinophilic gastritis 30.6 IL5 CCL26 CCL11
24 igg4-related disease 30.6 PRTN3 MPO IL5 IL10
25 neuritis 30.6 TNF IL1B IL10 CXCL8
26 endocarditis 30.6 TNF IL17A IL10 CXCL8
27 scleritis 30.6 TNF PRTN3 MPO IL5
28 paragonimiasis 30.6 RNASE3 IL5 CCL17
29 granulomatosis with polyangiitis 30.6 TNF RNASE3 PRTN3 MPO IL5 IL17A
30 polyradiculoneuropathy 30.6 IL17A IL10 HLA-DRB1
31 retroperitoneal fibrosis 30.5 HLA-DRB1 CCL11
32 myocarditis 30.5 TNF IL2 IL1B IL17A IL10 CXCL8
33 goodpasture syndrome 30.5 PRTN3 MPO HLA-DRB1
34 eosinophilic pneumonia 30.5 RNASE3 IL5 IL3 IL2 CXCL8 CCL17
35 sclerosing cholangitis 30.5 TNF IL17A IL10
36 proctitis 30.4 TNF RNASE3 MPO
37 loeffler endocarditis 30.4 THBD RNASE3 PRG2 IL5 IL3
38 autoimmune vasculitis 30.4 TNF PRTN3 MPO IL2 IL17A
39 inflammatory bowel disease 1 30.4 TNF IL1B IL17A IL10
40 invasive aspergillosis 30.4 TNF IL17A IL10
41 cryptococcosis 30.4 TNF IL17A IL10
42 henoch-schoenlein purpura 30.4 TNF RNASE3 PRTN3 MPO
43 cough variant asthma 30.4 RNASE3 IL5
44 infective endocarditis 30.4 PRTN3 MPO CXCL8
45 choroiditis 30.3 TNF IL17A IL10
46 peripheral nervous system disease 30.3 TNF PRTN3 MPO IL2 IL1B IL17A
47 takayasu arteritis 30.3 TNF THBD IL10 HLA-DRB1 HLA-B
48 human cytomegalovirus infection 30.3 TNF IL17A IL10
49 severe cutaneous adverse reaction 30.3 TNF HLA-DRB1 HLA-B
50 rhinitis 30.3 TNF RNASE3 PRG2 IL5 IL1B IL17A

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 30 (show top 50) (show all 54)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 sinusitis 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000246
2 congestive heart failure 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001635
3 autoimmunity 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002960
4 purpura 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000979
5 asthma 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002099
6 peripheral neuropathy 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0009830
7 weight loss 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001824
8 urticaria 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001025
9 vasculitis 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002633
10 central nervous system degeneration 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0007009
11 eosinophilia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001880
12 pulmonary infiltrates 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002113
13 nausea and vomiting 58 30 Frequent (33%) Frequent (79-30%)
HP:0002017
14 gait disturbance 58 30 Frequent (33%) Frequent (79-30%)
HP:0001288
15 dysphagia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002015
16 hypertension 58 30 Frequent (33%) Frequent (79-30%)
HP:0000822
17 fatigue 58 30 Frequent (33%) Frequent (79-30%)
HP:0012378
18 hypertrophic cardiomyopathy 58 30 Frequent (33%) Frequent (79-30%)
HP:0001639
19 venous thrombosis 58 30 Frequent (33%) Frequent (79-30%)
HP:0004936
20 hematuria 58 30 Frequent (33%) Frequent (79-30%)
HP:0000790
21 abdominal pain 58 30 Frequent (33%) Frequent (79-30%)
HP:0002027
22 arthralgia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002829
23 hypopigmented skin patches 58 30 Frequent (33%) Frequent (79-30%)
HP:0001053
24 skin rash 58 30 Frequent (33%) Frequent (79-30%)
HP:0000988
25 abnormal pericardium morphology 58 30 Frequent (33%) Frequent (79-30%)
HP:0001697
26 tubulointerstitial nephritis 58 30 Frequent (33%) Frequent (79-30%)
HP:0001970
27 abnormal pleura morphology 30 Frequent (33%) HP:0002103
28 respiratory insufficiency 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002093
29 arthritis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001369
30 malabsorption 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002024
31 gastroesophageal reflux 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002020
32 proteinuria 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000093
33 renal insufficiency 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000083
34 fever 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001945
35 cranial nerve paralysis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0006824
36 acrocyanosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001063
37 myocardial infarction 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001658
38 hemiplegia/hemiparesis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0004374
39 subcutaneous nodule 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001482
40 transient ischemic attack 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002326
41 myalgia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003326
42 glomerulopathy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100820
43 hemoptysis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002105
44 cough 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012735
45 myocarditis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012819
46 endocarditis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100584
47 recurrent intrapulmonary hemorrhage 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0006535
48 intestinal obstruction 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0005214
49 nasal polyposis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100582
50 papule 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0200034

Symptoms:

11
  • asthma

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

25 (show all 40)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 10.13 CXCL8 IL10 IL17A IL1B IL2RA MPO
2 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 10.13 CXCL8 IL10 IL17A IL1B IL2 IL2RA
3 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.96 IL10
4 Increased shRNA abundance (Z-score > 2) GR00366-A-104 9.96 IL2RA
5 Increased shRNA abundance (Z-score > 2) GR00366-A-109 9.96 HMGB1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-113 9.96 IL10
7 Increased shRNA abundance (Z-score > 2) GR00366-A-115 9.96 IL2RA
8 Increased shRNA abundance (Z-score > 2) GR00366-A-119 9.96 PRTN3
9 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.96 IL2RA
10 Increased shRNA abundance (Z-score > 2) GR00366-A-125 9.96 IL10
11 Increased shRNA abundance (Z-score > 2) GR00366-A-130 9.96 IL2RA
12 Increased shRNA abundance (Z-score > 2) GR00366-A-133 9.96 PRTN3
13 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.96 IL2RA
14 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.96 IL10 PRTN3
15 Increased shRNA abundance (Z-score > 2) GR00366-A-153 9.96 HMGB1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.96 IL10
17 Increased shRNA abundance (Z-score > 2) GR00366-A-163 9.96 HMGB1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-174 9.96 IL2RA
19 Increased shRNA abundance (Z-score > 2) GR00366-A-176 9.96 HMGB1
20 Increased shRNA abundance (Z-score > 2) GR00366-A-179 9.96 HMGB1 PRTN3
21 Increased shRNA abundance (Z-score > 2) GR00366-A-181 9.96 HMGB1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-198 9.96 IL10
23 Increased shRNA abundance (Z-score > 2) GR00366-A-202 9.96 IL2RA
24 Increased shRNA abundance (Z-score > 2) GR00366-A-209 9.96 PRTN3
25 Increased shRNA abundance (Z-score > 2) GR00366-A-210 9.96 IL10
26 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.96 IL10
27 Increased shRNA abundance (Z-score > 2) GR00366-A-25 9.96 IL2RA
28 Increased shRNA abundance (Z-score > 2) GR00366-A-34 9.96 IL2RA
29 Increased shRNA abundance (Z-score > 2) GR00366-A-46 9.96 IL2RA
30 Increased shRNA abundance (Z-score > 2) GR00366-A-52 9.96 IL10 IL2RA
31 Increased shRNA abundance (Z-score > 2) GR00366-A-53 9.96 IL10
32 Increased shRNA abundance (Z-score > 2) GR00366-A-6 9.96 HMGB1
33 Increased shRNA abundance (Z-score > 2) GR00366-A-65 9.96 IL10
34 Increased shRNA abundance (Z-score > 2) GR00366-A-67 9.96 IL10 IL2RA
35 Increased shRNA abundance (Z-score > 2) GR00366-A-68 9.96 IL10
36 Increased shRNA abundance (Z-score > 2) GR00366-A-69 9.96 HMGB1
37 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.96 HMGB1
38 Increased shRNA abundance (Z-score > 2) GR00366-A-82 9.96 IL10 PRTN3
39 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.96 IL10
40 Increased shRNA abundance (Z-score > 2) GR00366-A-99 9.96 PRTN3

MGI Mouse Phenotypes related to Churg-Strauss Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.06 CCL11 CCL17 HLA-B HMGB1 IL10 IL17A
2 digestive/alimentary MP:0005381 9.86 HLA-B IL10 IL17A IL2 IL2RA IL5
3 respiratory system MP:0005388 9.7 CCL11 HMGB1 IL10 IL17A IL2 IL2RA
4 hematopoietic system MP:0005397 9.55 CCL11 CCL17 CCL26 HLA-B HMGB1 IL10

Drugs & Therapeutics for Churg-Strauss Syndrome

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

(show top 50) (show all 72)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Levoleucovorin Approved, Experimental, Investigational Phase 4 68538-85-2, 58-05-9, 73951-54-9 149436 6006
3
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 4112 126941
4
Mycophenolic acid Approved, Investigational Phase 4 24280-93-1 446541
5
Azathioprine Approved Phase 4 446-86-6 2265
6
Rituximab Approved Phase 4 174722-31-7
7
Povidone K30 Approved, Experimental Phase 4 9003-39-8 6917 131751496
8
Hydroxychloroquine Approved Phase 4 118-42-3 3652
9
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
10 Dermatologic Agents Phase 4
11 Folic Acid Antagonists Phase 4
12 Folate Phase 4
13 Vitamin B9 Phase 4
14 Vitamin B Complex Phase 4
15 Gastrointestinal Agents Phase 4
16 Antiemetics Phase 4
17 Antitubercular Agents Phase 4
18 Protective Agents Phase 4
19 Antibiotics, Antitubercular Phase 4
20 Anti-Bacterial Agents Phase 4
21 Neuroprotective Agents Phase 4
22 Antirheumatic Agents Phase 4
23 Antimetabolites Phase 4
24 Immunosuppressive Agents Phase 4
25 Immunologic Factors Phase 4
26 Antineoplastic Agents, Immunological Phase 4
27 glucocorticoids Phase 4
28 Anti-Infective Agents Phase 4
29 Antimalarials Phase 4
30 Antiprotozoal Agents Phase 4
31 Antiparasitic Agents Phase 4
32
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
33
Coenzyme M Approved, Investigational Phase 3 3375-50-6 598 23662354
34
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
35
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
36
Prednisolone Approved, Vet_approved Phase 3 50-24-8 4894 5755
37
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5 1875
38
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 4159 6741
39
Benralizumab Approved, Investigational Phase 3 1044511-01-4
40
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7 4897
41 Antineoplastic Agents, Alkylating Phase 3
42 Alkylating Agents Phase 3
43 Antineoplastic Agents, Hormonal Phase 3
44 Hormones Phase 3
45 Hormone Antagonists Phase 3
46 Anti-Inflammatory Agents Phase 3
47
Methylprednisolone Acetate Phase 3 584547
48 Respiratory System Agents Phase 3
49 Anti-Asthmatic Agents Phase 3
50 Pharmaceutical Solutions Phase 3

Interventional clinical trials:

(show all 42)
# 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 Hydroxychloroquine in ANCA Vasculitis Evaluation - A Multicentre, Randomised, Double-blind, Placebo-controlled Trial Recruiting NCT04316494 Phase 4 Hydroxychloroquine;Placebo
4 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
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 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
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 Completed NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
8 Evaluation of MEpolizumab-based Regimen Compared to Conventional Therapeutic Strategy For Remission Induction In Patients With Eosinophilic Granulomatosis With Polyangiitis. Prospective, Randomized, Controlled, Double-blind Study Recruiting NCT05030155 Phase 3 Mepolizumab;cyclophosphamide/azathioprine;Placebo
9 A 52-week, Randomized, Double-blind, Double-dummy, Parallel-group, Multi-centre, Non-inferiority Study to Investigate the Efficacy and Safety of Depemokimab Compared With Mepolizumab in Adults With Relapsing or Refractory Eosinophilic Granulomatosis With Polyangiitis (EGPA) Receiving Standard of Care (SoC) Therapy Recruiting NCT05263934 Phase 3 Placebo matching mepolizumab;Placebo matching depemokimab
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 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 Active, not recruiting NCT04157348 Phase 3
12 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
13 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
14 Mepolizumab As a Steroid Sparing Treatment Option in the Churg Strauss Syndrome Completed NCT00527566 Phase 1, Phase 2
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 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 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
18 The Efficacy and Safety of Benralizumab In the Treatment of Eosinophilic Grandulomatosis With Polyangiitis (EGPA) Study: BITE Active, not recruiting NCT03010436 Phase 2 Benralizumab
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 Eosinophilic Granulomatosis With Polyangiitis Cohort Unknown status NCT03036670
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 VCRC Validation of Patient-Reported Diagnostic Data Completed NCT02190942
25 Illness Perception, Fatigue, and Function in Systemic Vasculitis (The VCRC Vasculitis Perception (VIP) Study) Completed NCT02190916
26 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
27 Infliximab Versus Rituximab in Systemic Necrotizing Vasculitides With Positive ANCA After Relapse or Resistant Immunosuppressant Therapies Completed NCT00307593 Infliximab;Rituximab
28 Educational Needs of Patients With Systemic Vasculitis- an International Survey Completed NCT02190929
29 The ANCA Vasculitis Questionnaire (AAV-PRO©) Completed NCT02507024
30 The Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
31 The Vasculitis Pregnancy Registry (V-PREG) Recruiting NCT02593565
32 Identification of Autoantigens and Their Potential Post-translational Modification in EGPA and Severe Eosinophilic Asthma Recruiting NCT04671446
33 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Recruiting NCT02006134
34 Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Recruiting NCT00315380
35 VCRC Tissue Biorepository Collection Protocol Recruiting NCT02967068
36 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
37 VCRC Genetic Repository One-Time DNA Protocol Recruiting NCT01241305
38 Prospective Registry of Eosinophilia With Respiratory Manifestations With Translational Research Identifying and Characterizing Eosinophils Recruiting NCT04538937
39 NUCALA ® Subcutaneous Injection Special Drug Use Investigation (EGPA, Long-term) Active, not recruiting NCT03557060 Nucala
40 A Single Arm, Multi-center Study to Assess the Long-term Real-world Safety and Effectiveness of Nucala in EGPA Patients Who Have Already Used Nucala for at Least 96 Weeks in Japan Active, not recruiting NCT04551989
41 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") Active, not recruiting NCT04280601
42 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

Organs/tissues related to Churg-Strauss Syndrome:

MalaCards : Lung, Bone, Neutrophil, Skin, Heart, Small Intestine, T Cells

Publications for Churg-Strauss Syndrome

Articles related to Churg-Strauss Syndrome:

(show top 50) (show all 3185)
# Title Authors PMID Year
1
IL-5 pathway inhibition in the treatment of asthma and Churg-Strauss syndrome. 53 62
20513522 2010
2
A comparative study of the diagnostic accuracy of ELISA systems for the detection of anti-neutrophil cytoplasm antibodies available in Japan and Europe. 53 62
19210866 2008
3
Pathways to ANCA production: from differentiation of dendritic cells by proteinase 3 to B lymphocyte maturation in Wegener's granuloma. 53 62
18181035 2008
4
Antibodies to selected minor target antigens in patients with anti-neutrophil cytoplasmic antibodies (ANCA). 53 62
17614969 2007
5
Meta-analysis of myeloperoxidase G-463/A polymorphism in anti-neutrophil cytoplasmic autoantibody-positive vasculitis. 53 62
17521322 2007
6
[Classification of systemic vasculatides]. 53 62
17408915 2007
7
Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. 53 62
17143091 2007
8
TNF-alpha bioactivity-inhibiting therapy in ANCA-associated vasculitis: clinical and experimental considerations. 53 62
17699331 2006
9
Prevalence of antineutrophil cytoplasmic antibodies in patients with various pulmonary diseases or multiorgan dysfunction. 53 62
12687504 2003
10
Autoantibodies in vasculitis. 53 62
12723981 2003
11
Benefit-risk assessment of antileukotrienes in the management of asthma. 53 62
12735786 2003
12
[Microscopic polyangiitis]. 53 62
10896971 2000
13
Distinct differences in autoantigen specificity of anti-neutrophil cytoplasm antibodies in systemic vasculitides and other inflammatory diseases. 53 62
11370121 1999
14
[Leprous neuropathy--observation from the standpoint vasculitis]. 53 62
10481446 1999
15
Pulmonary eosinophilia associated with montelukast. 53 62
10335014 1999
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What is new with anti-neutrophil cytoplasmic antibodies: diagnostic, pathogenetic and therapeutic implications. 53 62
10456261 1999
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[Clinical study on five myeloperoxidase specific anti-neutrophil cytoplasmic antibody (MPO-ANCA) positive Churg-Strauss syndrome cases]. 53 62
9852746 1998
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Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. 53 62
9506572 1998
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[Eosinophil cationic protein in a 39-year-old patient with Churg-Strauss syndrome]. 53 62
9465849 1998
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Serum interleukin-5 levels in a case with allergic granulomatous angiitis. 53 62
9372332 1997
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Systemic necrotizing vasculitis. 53 62
9220078 1997
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[A case of Churg-Strauss syndrome in which MPO-ANCA (antibodies to myeloperoxidase) appeared to reflect the disease activity]. 53 62
7609341 1995
23
[Autoantibodies associated with vasculitis]. 53 62
7933581 1994
24
Correlation of antineutrophil cytoplasmic antibodies with the extrarenal histopathology of Wegener's (pathergic) granulomatosis and related forms of vasculitis. 53 62
8381764 1993
25
Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. 53 62
1466365 1992
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[Antineutrophil cytoplasmic antibodies--ANCA]. 53 62
1464072 1992
27
Detection of autoantibodies against myeloid lysosomal enzymes: a useful adjunct to classification of patients with biopsy-proven necrotizing arteritis. 53 62
1677531 1991
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At the Heart of Eosinophilic Granulomatosis with Polyangiitis: into Cardiac and Vascular Involvement. 62
36194339 2022
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The Role of T Helper Type 2 (Th2) Cytokines in the Pathogenesis of Eosinophilic Granulomatosis with Polyangiitis (eGPA): an Illustrative Case and Discussion. 62
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Multi-targeted therapy for refractory eosinophilic granulomatosis with polyangiitis characterized by intracerebral hemorrhage and cardiomyopathy: a case-based review. 62
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Effect of Dupilumab on Blood Eosinophil Counts in Patients With Asthma, Chronic Rhinosinusitis With Nasal Polyps, Atopic Dermatitis, or Eosinophilic Esophagitis. 62
35636689 2022
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Eosinophilic Granulomatosis With Polyangiitis With a Pathologically Proven Calcified Lung Nodule. 62
36182108 2022
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ANCA-associated vasculitis with protein-losing enteropathy is characterized by hypocomplementemia. 62
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ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis. 62
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Clinical Images: Dacryoadenitis in eosinophilic granulomatosis with polyangiitis mimicking immunoglobulin G4-related disease. 62
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Eosinophilic Granulomatosis with Polyangiitis. 62
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Radiological significance of mediastinal lymphadenopathy in eosinophilic granulomatosis with polyangiitis. 62
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[Recommendations on the diagnosis and treatment of anti-neutrophil cytoplasmic antibody associated vasculitis in China]. 62
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Relapsing subarachnoid hemorrhage as a clinical manifestation in microscopic polyangiitis: a case report and literature review. 62
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Structured histopathology and laboratory evidence in nasal polyposis with different pathogenesis. 62
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What's behind your eosinophilic myocarditis? A case of Churg-Strauss syndrome diagnosed during acute heart failure. 62
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Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management. 62
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Case report of eosinophilic granulomatosis with polyangitis presenting as acute myocarditis. 62
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Targeting eosinophils in chronic respiratory diseases using nanotechnology-based drug delivery. 62
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[Efficacy of omalizumab in the treatment of eosinophilic granulomatous polyangiitis with asthma as the first symptom]. 62
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Optimal management of ANCA-associated vasculitis before and during pregnancy: current perspectives. 62
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Long-term Safety and Efficacy of Benralizumab for Eosinophilic Granulomatosis with Polyangiitis Complicated with Severe Neuropathy. 62
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A diagnostic score for eosinophilic granulomatosis with polyangiitis among eosinophilic disorders. 62
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New-onset eosinophilic granulomatosis with polyangiitis in 2 patients during treatment with IL-5 pathway inhibitors. 62
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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 top 50) (show all 57)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.85 CCL11 CXCL8 HLA-B HLA-DRB1 HMGB1 IL10
2
Show member pathways
13.84 TNF PRG2 IL5 IL3 IL2RA IL2
3
Show member pathways
13.68 CCL11 CCL17 CCL26 CXCL8 IL10 IL17A
4
Show member pathways
13.56 TNF IL5 IL3 IL2RA IL2 IL1B
5
Show member pathways
13.44 TNF IL5 IL3 IL2RA IL2 IL1B
6
Show member pathways
13.3 CCL11 CCL17 CCL26 CXCL8 IL10 IL17A
7
Show member pathways
13.18 CCL11 CXCL8 HLA-B HLA-DRB1 HMGB1 IL10
8
Show member pathways
12.84 TNF IL2RA IL2 IL1B CXCL8
9
Show member pathways
12.68 TNF IL5 IL3 IL2RA IL2 IL1B
10
Show member pathways
12.65 TNF MPO IL2 IL1B IL17A IL10
11 12.64 IL10 IL17A IL1B IL2 IL2RA IL3
12
Show member pathways
12.43 IL5 IL2RA IL2 IL1B
13
Show member pathways
12.41 TNF IL1B CXCL8 CCL11
14
Show member pathways
12.24 TNF IL5 IL2 IL17A IL10 HLA-DRB1
15
Show member pathways
12.22 HLA-B IL2 IL3 TNF
16
Show member pathways
12.21 IL5 IL3 IL2RA IL2
17 12.19 TNF IL1B IL10 CXCL8
18
Show member pathways
12.09 CXCL8 CCL26 CCL17 CCL11
19
Show member pathways
12.06 IL5 IL2RA IL2 IL1B
20 12.05 TNF IL1B IL17A IL10 CXCL8 CCL11
21 12.04 TNF MPO IL5 IL3 IL2RA IL2
22 12.03 TNF IL2 IL1B CXCL8
23
Show member pathways
12.02 IL10 IL17A IL1B IL2 TNF
24 11.87 TNF THBD IL2 IL1B IL10
25 11.84 TNF IL1B HMGB1 CXCL8
26 11.8 IL2RA IL2 IL1B IL17A
27
Show member pathways
11.8 TNF IL5 IL3 IL2RA IL2 CXCL8
28 11.79 IL5 IL2 IL10 CXCL8
29 11.77 IL5 IL2 CXCL8
30 11.77 TNF IL5 IL1B CXCL8 CCL11
31 11.77 TNF IL5 IL2RA IL2 IL1B IL17A
32
Show member pathways
11.75 TNF IL2 IL1B CXCL8
33 11.74 IL10 IL2 TNF
34 11.74 CCL11 CCL17 CCL26 IL10 IL5
35
Show member pathways
11.71 IL10 IL2 IL5 TNF
36 11.68 TNF IL10 CXCL8
37 11.67 TNF IL1B IL10
38 11.63 TNF IL2RA IL2 IL10 HLA-DRB1
39 11.61 TNF IL1B IL10 CXCL8
40 11.59 TNF IL10 CXCL8
41 11.54 IL5 IL2RA IL2 IL10 CCL17 CCL11
42
Show member pathways
11.48 TNF IL2RA IL2 IL10
43 11.42 TNF IL5 IL1B IL17A
44 11.41 TNF IL1B IL10
45 11.38 IL5 IL2RA IL2
46 11.35 TNF IL1B IL17A CXCL8
47 11.33 TNF IL1B CXCL8
48 11.29 TNF IL5 IL2RA IL2 IL1B IL17A
49 11.26 IL5 IL2 IL10
50 11.04 IL1B MPO TNF

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 10.15 CCL11 CCL17 CCL26 CXCL8 HMGB1 IL10
2 extracellular space GO:0005615 9.85 CCL11 CCL17 CCL26 CXCL8 HLA-DRB1 HMGB1

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

(show all 39)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.61 CCL11 CCL26 CXCL8 HLA-DRB1 HMGB1 IL10
2 cellular response to tumor necrosis factor GO:0071356 10.27 CCL11 CCL17 CCL26 CXCL8
3 defense response to Gram-positive bacterium GO:0050830 10.25 TNF RNASE3 IL1B IL17A
4 positive regulation of DNA-binding transcription factor activity GO:0051091 10.25 TNF IL5 IL1B IL10
5 defense response to bacterium GO:0042742 10.22 IL10 MPO PRG2 RNASE3 TNF
6 positive regulation of interleukin-6 production GO:0032755 10.22 TNF IL1B IL17A HMGB1
7 cellular response to type II interferon GO:0071346 10.19 TNF CCL26 CCL17 CCL11
8 cell-cell signaling GO:0007267 10.18 IL3 IL2 IL1B IL17A CCL26 CCL17
9 monocyte chemotaxis GO:0002548 10.16 CCL26 CCL17 CCL11
10 cellular response to lipopolysaccharide GO:0071222 10.16 TNF IL1B IL10 HMGB1 CXCL8
11 negative regulation of T cell proliferation GO:0042130 10.15 IL2RA IL10 HLA-DRB1
12 negative regulation of type II interferon production GO:0032689 10.15 IL10 HMGB1 HLA-DRB1
13 positive regulation of ERK1 and ERK2 cascade GO:0070374 10.15 CCL11 CCL17 CCL26 HLA-DRB1 HMGB1 TNF
14 defense response GO:0006952 10.12 CXCL8 HLA-B MPO TNF
15 positive regulation of receptor signaling pathway via JAK-STAT GO:0046427 10.11 TNF IL5 IL10
16 lymphocyte chemotaxis GO:0048247 10.1 CCL26 CCL17 CCL11
17 chemokine-mediated signaling pathway GO:0070098 10.1 CXCL8 CCL26 CCL17 CCL11
18 positive regulation of activated T cell proliferation GO:0042104 10.07 IL2RA IL2 HMGB1
19 response to lipopolysaccharide GO:0032496 10.06 TNF THBD MPO IL1B IL10
20 positive regulation of immunoglobulin production GO:0002639 10.05 IL5 IL2 IL10
21 negative regulation of neurogenesis GO:0050768 10.04 TNF IL1B CCL11
22 inflammatory response to antigenic stimulus GO:0002437 10.03 HLA-DRB1 HMGB1 IL2RA
23 immune system process GO:0002376 10.02 HLA-B HLA-DRB1 HMGB1 IL17A IL2 IL2RA
24 neutrophil chemotaxis GO:0030593 10.02 CCL11 CCL17 CCL26 CXCL8 IL1B
25 negative regulation of cytokine production involved in immune response GO:0002719 9.96 TNF IL10
26 positive regulation of fever generation GO:0031622 9.96 TNF IL1B
27 cellular response to interleukin-1 GO:0071347 9.96 CCL11 CCL17 CCL26 CXCL8 IL17A
28 inflammatory response GO:0006954 9.96 CCL11 CCL17 CCL26 CXCL8 HMGB1 IL17A
29 sequestering of triglyceride GO:0030730 9.93 TNF IL1B
30 regulation of T cell homeostatic proliferation GO:0046013 9.91 IL2 IL2RA
31 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.91 IL1B TNF
32 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.91 TNF IL1B IL10
33 positive regulation of plasma cell differentiation GO:1900100 9.9 IL10 IL2
34 chemotaxis GO:0006935 9.9 RNASE3 HMGB1 CXCL8 CCL26 CCL17 CCL11
35 negative regulation of lymphocyte proliferation GO:0050672 9.85 IL2RA IL2
36 regulation of CD4-positive, alpha-beta T cell proliferation GO:2000561 9.85 IL2RA IL2
37 response to gold nanoparticle GO:1990268 9.84 TNF MPO
38 chronic inflammatory response to antigenic stimulus GO:0002439 9.83 TNF IL10
39 immune response GO:0006955 9.7 TNF PRG2 IL5 IL3 IL2RA IL2

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

# Name GO ID Score Top Affiliating Genes
1 CCR chemokine receptor binding GO:0048020 9.63 CCL26 CCL17 CCL11
2 cytokine activity GO:0005125 9.62 TNF IL5 IL3 IL2 IL1B IL17A
3 CCR3 chemokine receptor binding GO:0031728 9.56 CCL26 CCL11
4 chemokine activity GO:0008009 9.56 CXCL8 CCL26 CCL17 CCL11

Sources for Churg-Strauss Syndrome

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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