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 20 58 54 44 15 17 71
Eosinophilic Granulomatosis with Polyangiitis 20 58 36
Granulomatous Allergic Angiitis 20 58
Churg-Strauss Vasculitis 12 20
Egpa 20 58
Allergic Angiitis and Granulomatosis 20
Allergic Granulomatous and Angiitis 20
Allergic Granulomatosis Angiitis 12
Allergic Granulomatous Angiitis 12
Allergic Granulomatosis 20
Css 20

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 vasculitis, 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 Azathioprine and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include neutrophil, lung and skin, and related phenotypes are sinusitis and congestive heart failure

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.

GARD : 20 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 873)
# Related Disease Score Top Affiliating Genes
1 microscopic polyangiitis 32.7 MPO HLA-DRB1
2 vasculitis 32.0 TNF THBD PRTN3 MPO IL17A
3 granulomatous angiitis 31.7 PRTN3 IL5
4 polyarteritis nodosa 31.7 PRTN3 MPO HLA-DRB1
5 mononeuritis multiplex 31.5 TNF PRTN3 MPO IL5 CCL26
6 granulomatosis with polyangiitis 31.4 TNF PRTN3 MPO IL5 CCL26
7 mononeuropathy 31.4 TNF PRTN3 MPO
8 chronic eosinophilic pneumonia 31.2 RNASE3 IL5 CCL11
9 purpura 31.1 TNF THBD IL1B IL10
10 maxillary sinusitis 31.1 RNASE3 PRTN3 MPO
11 ige responsiveness, atopic 31.1 RNASE3 IL5 IL10
12 anca-associated vasculitis 31.0 PRTN3 MPO IL2
13 pericarditis 30.9 TNF IL1B CXCL8
14 dacryoadenitis 30.8 PRTN3 IL17A IL10
15 cellulitis 30.8 TNF RNASE3 IL5
16 endomyocardial fibrosis 30.7 TNF RNASE3 PRG2 IL5 IL10
17 allergic bronchopulmonary aspergillosis 30.7 IL5 IL10 HLA-DRB1 CCL17
18 eosinophilic pneumonia 30.7 RNASE3 IL5 IL3 CXCL8 CCL17 CCL11
19 guillain-barre syndrome 30.7 TNF IL17A HLA-DRB1
20 chronic rhinitis 30.7 RNASE3 PRG2 MPO IL5
21 autoimmune inner ear disease 30.7 TNF MPO
22 eosinophilic gastritis 30.6 IL5 CCL26 CCL11
23 hypereosinophilic syndrome, idiopathic 30.6 IL5 IL3
24 acute asthma 30.6 RNASE3 IL5
25 myocarditis 30.6 TNF IL2 IL1B IL17A IL10
26 multifocal choroiditis 30.6 TNF IL10
27 retroperitoneal fibrosis 30.6 HLA-DRB1 CCL11
28 endocarditis 30.5 TNF IL17A IL10 CXCL8
29 henoch-schoenlein purpura 30.5 RNASE3 PRTN3 MPO
30 scleritis 30.5 TNF PRTN3 MPO IL5
31 allergic rhinitis 30.5 RNASE3 MPO IL5 IL17A IL10 CXCL8
32 goodpasture syndrome 30.5 PRTN3 MPO HLA-DRB1
33 intrinsic asthma 30.4 RNASE3 IL5 IL3
34 autoimmune hepatitis 30.4 TNF IL17A IL10 HLA-DRB1
35 hypersensitivity vasculitis 30.4 TNF PRTN3 MPO
36 proctitis 30.4 TNF RNASE3 MPO
37 urticaria 30.4 TNF RNASE3 PRG2 IL5
38 neuritis 30.4 TNF IL1B IL10 HLA-DRB1 CXCL8
39 ileus 30.4 TNF IL1B IL10
40 cryptococcosis 30.4 TNF IL17A IL10
41 respiratory allergy 30.4 RNASE3 IL5 IL3 IL10
42 cryoglobulinemia 30.4 TNF IL1B HLA-DRB1
43 eosinophilic gastroenteritis 30.4 RNASE3 PRG2 IL5 IL3 CCL26 CCL11
44 enterocolitis 30.3 TNF IL10 CXCL8
45 gastric ulcer 30.3 TNF IL1B CXCL8
46 choroiditis 30.3 TNF IL17A IL10
47 chronic asthma 30.3 RNASE3 IL5 IL2 CCL11
48 peripheral nervous system disease 30.3 TNF IL2 IL1B IL17A IL10 CXCL8
49 recurrent respiratory papillomatosis 30.3 IL2 HLA-DRB1
50 takayasu arteritis 30.2 TNF THBD IL10 HLA-DRB1

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 sinusitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000246
2 congestive heart failure 58 31 hallmark (90%) Very frequent (99-80%) HP:0001635
3 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
4 purpura 58 31 hallmark (90%) Very frequent (99-80%) HP:0000979
5 asthma 58 31 hallmark (90%) Very frequent (99-80%) HP:0002099
6 peripheral neuropathy 58 31 hallmark (90%) Very frequent (99-80%) HP:0009830
7 weight loss 58 31 hallmark (90%) Very frequent (99-80%) HP:0001824
8 urticaria 58 31 hallmark (90%) Very frequent (99-80%) HP:0001025
9 vasculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002633
10 central nervous system degeneration 58 31 hallmark (90%) Very frequent (99-80%) HP:0007009
11 eosinophilia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001880
12 pulmonary infiltrates 58 31 hallmark (90%) Very frequent (99-80%) HP:0002113
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 hypertrophic cardiomyopathy 58 31 frequent (33%) Frequent (79-30%) HP:0001639
19 venous thrombosis 58 31 frequent (33%) Frequent (79-30%) HP:0004936
20 hematuria 58 31 frequent (33%) Frequent (79-30%) HP:0000790
21 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
22 arthralgia 58 31 frequent (33%) Frequent (79-30%) HP:0002829
23 hypopigmented skin patches 58 31 frequent (33%) Frequent (79-30%) HP:0001053
24 skin rash 58 31 frequent (33%) Frequent (79-30%) HP:0000988
25 abnormal pericardium morphology 58 31 frequent (33%) Frequent (79-30%) HP:0001697
26 tubulointerstitial nephritis 58 31 frequent (33%) Frequent (79-30%) HP:0001970
27 abnormal pleura morphology 31 frequent (33%) HP:0002103
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 acrocyanosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001063
37 myocardial infarction 58 31 occasional (7.5%) Occasional (29-5%) HP:0001658
38 hemiplegia/hemiparesis 58 31 occasional (7.5%) Occasional (29-5%) HP:0004374
39 subcutaneous nodule 58 31 occasional (7.5%) Occasional (29-5%) HP:0001482
40 transient ischemic attack 58 31 occasional (7.5%) Occasional (29-5%) HP:0002326
41 myalgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003326
42 glomerulopathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0100820
43 hemoptysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002105
44 cough 58 31 occasional (7.5%) Occasional (29-5%) HP:0012735
45 myocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012819
46 endocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100584
47 recurrent intrapulmonary hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0006535
48 intestinal obstruction 58 31 occasional (7.5%) Occasional (29-5%) HP:0005214
49 nasal polyposis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100582
50 papule 58 31 occasional (7.5%) Occasional (29-5%) HP:0200034

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 9.44 CXCL8 IL10 IL17A IL1B MPO TNF
40 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.44 CXCL8 IL10 IL17A IL1B IL2 MPO

MGI Mouse Phenotypes related to Churg-Strauss Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.93 CCL11 CCL17 IL10 IL17A IL1B IL2
2 immune system MP:0005387 9.77 CCL11 CCL17 CYSLTR1 IL10 IL17A IL1B
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 72)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Azathioprine Approved Phase 4 446-86-6 2265
2
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
3
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
4 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
6
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
7
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
8
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
9
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
10
Levoleucovorin Approved, Investigational Phase 4 68538-85-2 149436
11
Mycophenolic acid Approved Phase 4 24280-93-1 446541
12
Povidone Approved Phase 4 9003-39-8 131751496
13
Hydroxychloroquine Approved Phase 4 118-42-3 3652
14
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
15
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
16 Immunologic Factors Phase 4
17 Alkylating Agents Phase 4
18 Anti-Inflammatory Agents Phase 4
19 Hormones Phase 4
20 Hormone Antagonists Phase 4
21 glucocorticoids Phase 4
22 Methylprednisolone Acetate Phase 4
23 Antineoplastic Agents, Hormonal Phase 4
24 Antibiotics, Antitubercular Phase 4
25 Gastrointestinal Agents Phase 4
26 Folic Acid Antagonists Phase 4
27 Protective Agents Phase 4
28 Antitubercular Agents Phase 4
29 Vitamin B Complex Phase 4
30 Neuroprotective Agents Phase 4
31 Anti-Bacterial Agents Phase 4
32 Vitamin B9 Phase 4
33 Dermatologic Agents Phase 4
34 Folate Phase 4
35 Antiemetics Phase 4
36 Immunosuppressive Agents Phase 4
37 Antirheumatic Agents Phase 4
38 Antimetabolites Phase 4
39 Anti-Infective Agents Phase 4
40 Antiparasitic Agents Phase 4
41 Antiprotozoal Agents Phase 4
42 Antimalarials Phase 4
43 Benralizumab Approved, Investigational Phase 3 1044511-01-4
44 Respiratory System Agents Phase 3
45 Anti-Asthmatic Agents Phase 3
46 Pharmaceutical Solutions Phase 3
47
Reslizumab Approved, Investigational Phase 2 241473-69-8
48
Naltrexone Approved, Investigational, Vet_approved Phase 2 16590-41-3 5360515
49
Ethanol Approved Phase 2 64-17-5 702
50
rituximab Approved Phase 2 174722-31-7 10201696

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 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 Recruiting NCT04316494 Phase 4 Hydroxychloroquine;Placebo
5 Evaluation of Rituximab-based Regimen Compared to Conventional Therapeutic Strategy For Remission Induction In Patients With Newly-Diagnosed or Relapsing Eosinophilic Granulomatosis With Polyangiitis. Prospective, Randomized, Controlled, Double-blind Study Unknown status NCT02807103 Phase 3 Rituximab;Placebo-rituximab;Cyclophosphamide;Placebo-cyclophosphamide
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 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
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 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
11 Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial. Unknown status NCT00751517 Phase 2 Methotrexate;Cyclophosphamide
12 The Efficacy and Safety of Benralizumab In the Treatment of Eosinophilic Grandulomatosis With Polyangiitis (EGPA) Study: BITE Unknown status NCT03010436 Phase 2 Benralizumab
13 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
14 Mepolizumab As a Steroid Sparing Treatment Option in the Churg Strauss Syndrome Completed NCT00527566 Phase 1, Phase 2
15 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
16 Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis Recruiting NCT03482479 Phase 2 Naltrexone Hydrochloride
17 A Pilot Study on the Use of Rituximab in the Treatment of Churg- Strauss Syndrome With Renal Involvement Terminated NCT00424749 Phase 2 Rituximab;Prednisone
18 Eosinophilic Granulomatosis With Polyangiitis Cohort Unknown status NCT03036670
19 Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases Unknown status NCT00006055 anti-thymocyte globulin;cyclophosphamide;cyclosporine;filgrastim;methylprednisolone;prednisone
20 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
21 ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis Unknown status NCT01066208
22 Neutrophils Function and Identification of Prognostic Factors in Granulomatosis With Polyangiitis (Formerly Named Wegener's Granulomatosis). Unknown status NCT01862068
23 The ANCA Vasculitis Questionnaire (AAV-PRO©) Completed NCT02507024
24 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
25 Infliximab Versus Rituximab in Systemic Necrotizing Vasculitides With Positive ANCA After Relapse or Resistant Immunosuppressant Therapies Completed NCT00307593 Infliximab;Rituximab
26 Illness Perception, Fatigue, and Function in Systemic Vasculitis (The VCRC Vasculitis Perception (VIP) Study) Completed NCT02190916
27 The Journey of Patients With Vasculitis From First Symptom to Diagnosis Completed NCT03410290
28 VCRC Validation of Patient-Reported Diagnostic Data Completed NCT02190942
29 Educational Needs of Patients With Systemic Vasculitis- an International Survey Completed NCT02190929
30 Soluble CD95 Ligand Role in the Pathophysiology of ANCA Associated Vasculitis Recruiting NCT03698071
31 Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Recruiting NCT00315380
32 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") Recruiting NCT04280601
33 Identification of Autoantigens and Their Potential Post-translational Modification in EGPA and Severe Eosinophilic Asthma Recruiting NCT04671446
34 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 Recruiting NCT04551989
35 Chronic Childhood Vasculitis: Characterizing the Individual Rare Diseases to Improve Patient Outcomes Recruiting NCT02006134
36 The Vasculitis Pregnancy Registry (V-PREG) Recruiting NCT02593565
37 VCRC Tissue Biorepository Collection Protocol Recruiting NCT02967068
38 Clinical Transcriptomics in Systemic Vasculitis (CUTIS) Recruiting NCT03004326
39 Prospective Registry of Eosinophilia With Respiratory Manifestations With Translational Research Identifying and Characterizing Eosinophils Recruiting NCT04538937
40 VCRC Genetic Repository One-Time DNA Protocol Active, not recruiting NCT01241305
41 NUCALA ® Subcutaneous Injection Special Drug Use Investigation (EGPA, Long-term) Active, not recruiting NCT03557060 Nucala
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

MalaCards organs/tissues related to Churg-Strauss Syndrome:

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

Publications for Churg-Strauss Syndrome

Articles related to Churg-Strauss Syndrome:

(show top 50) (show all 2575)
# Title Authors PMID Year
1
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. 61 20
28514601 2017
2
IL-5 pathway inhibition in the treatment of asthma and Churg-Strauss syndrome. 61 54
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. 61 54
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. 61 54
12723981 2003
12
Benefit-risk assessment of antileukotrienes in the management of asthma. 61 54
12735786 2003
13
[Microscopic polyangiitis]. 61 54
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
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Pulmonary eosinophilia associated with montelukast. 54 61
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What is new with anti-neutrophil cytoplasmic antibodies: diagnostic, pathogenetic and therapeutic implications. 61 54
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Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. 54 61
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[Eosinophil cationic protein in a 39-year-old patient with Churg-Strauss syndrome]. 61 54
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Systemic necrotizing vasculitis. 61 54
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[A case of Churg-Strauss syndrome in which MPO-ANCA (antibodies to myeloperoxidase) appeared to reflect the disease activity]. 54 61
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[Autoantibodies associated with vasculitis]. 61 54
7933581 1994
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Correlation of antineutrophil cytoplasmic antibodies with the extrarenal histopathology of Wegener's (pathergic) granulomatosis and related forms of vasculitis. 61 54
8381764 1993
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Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. 54 61
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[Antineutrophil cytoplasmic antibodies--ANCA]. 61 54
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Detection of autoantibodies against myeloid lysosomal enzymes: a useful adjunct to classification of patients with biopsy-proven necrotizing arteritis. 54 61
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A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Presenting as Ischemic Stroke and Splenic Infarction. 61
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Conjunctival Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) in a Young Adult Male With Persistent Ptosis. 61
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Eosinophilic Granulomatosis With Polyangiitis: An Unusual Case of Pediatric Subglottic Stenosis. 61
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Asystolic cardiac arrest with chest pain as initial features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). 61
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Biologics for Eosinophilic Granulomatosis with Polyangiitis: one size does not fit all. 61
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Case Report: A 64-Year-Old Man with 10-Year History of Eosinophilic Granulomatosis with Polyangiitis with Bronchiectasis and Severe Klebsiella pneumonia. 61
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Efficacy and Safety of Reslizumab in the Treatment of Eosinophilic Granulomatosis with Polyangiitis (EGPA). 61
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Polyangiitis overlap syndrome: a novel presentation of microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. 61
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Association Between IL-5 Levels and the Clinicopathologic Features of Eosinophilic Granulomatosis With Polyangiitis. 61
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Pathological entities that may affect the lungs and the myocardium. Evaluation with chest CT and cardiac MR. 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 top 50) (show all 56)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
14 TNF RNASE3 PRTN3 PRG2 MPO IL5
2
Show member pathways
13.88 TNF PRG2 IL5 IL3 IL2 IL1B
3
Show member pathways
13.73 TNF IL5 IL3 IL2 IL1B IL17A
4
Show member pathways
13.59 TNF IL5 IL3 IL2 IL1B IL17A
5
Show member pathways
13.41 TNF IL5 IL3 IL2 IL1B IL17A
6
Show member pathways
13.36 TNF PRTN3 IL5 IL3 IL2 IL1B
7
Show member pathways
13.18 TNF IL5 IL3 IL2 IL1B IL17A
8
Show member pathways
12.82 TNF IL2 IL1B HLA-DRB1 CXCL8
9
Show member pathways
12.68 TNF IL5 IL2 IL1B IL17A IL10
10
Show member pathways
12.67 TNF IL5 IL2 IL1B IL17A IL10
11
Show member pathways
12.66 TNF MPO IL2 IL1B IL17A IL10
12 12.63 TNF MPO IL3 IL2 IL1B IL17A
13
Show member pathways
12.41 IL5 IL3 IL2 IL10
14
Show member pathways
12.35 TNF MPO IL2 IL1B
15
Show member pathways
12.31 TNF IL5 IL1B IL17A CXCL8 CCL17
16
Show member pathways
12.3 TNF IL2 IL1B IL10 CCL17
17
Show member pathways
12.28 TNF IL1B IL10 HLA-DRB1
18
Show member pathways
12.28 TNF IL2 IL10 CXCL8 CCL26 CCL17
19 12.26 TNF IL1B IL10 HLA-DRB1
20
Show member pathways
12.21 TNF IL1B CXCL8 CCL11
21 12.2 TNF IL1B IL10 CXCL8
22 12.17 TNF IL2 IL1B IL10 CXCL8
23
Show member pathways
12.14 TNF RNASE3 PRG2 IL5 IL3 IL2
24
Show member pathways
12.13 TNF IL5 IL3 IL2 IL1B IL17A
25 12.06 TNF IL2 IL1B CXCL8
26 12.04 TNF MPO IL5 IL3 IL2 IL10
27 12.01 TNF IL5 IL3 IL1B HLA-DRB1
28 11.99 TNF IL1B IL10 CXCL8
29 11.99 TNF IL1B IL17A IL10 CXCL8 CCL11
30 11.97 TNF THBD IL1B CXCL8
31 11.97 TNF IL1B IL17A HLA-DRB1 CXCL8
32
Show member pathways
11.87 TNF IL2 IL1B IL17A IL10
33
Show member pathways
11.86 TNF IL5 IL3 IL2 CXCL8
34 11.85 TNF IL1B IL10 CXCL8
35 11.83 TNF THBD IL2 IL1B IL10
36 11.81 IL5 IL2 CXCL8
37
Show member pathways
11.76 TNF IL2 IL1B CXCL8
38 11.76 TNF IL5 IL1B CXCL8 CCL11
39 11.74 IL5 IL10 CCL26 CCL17 CCL11
40 11.71 TNF IL5 IL10
41 11.64 TNF IL1B CXCL8
42 11.64 TNF IL1B IL10 CXCL8
43 11.64 TNF IL5 IL2 IL1B IL17A IL10
44 11.63 TNF IL1B IL10 CXCL8
45 11.56 IL5 IL3 IL1B
46 11.56 TNF IL2 IL1B
47 11.54 IL5 IL2 IL10 CCL17 CCL11
48 11.43 TNF IL1B IL10
49 11.43 TNF IL5 IL1B IL17A
50
Show member pathways
11.42 TNF IL2 IL10

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 9.86 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 41)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 10.08 PRTN3 IL5 IL3 IL2 IL1B
2 neutrophil degranulation GO:0043312 10.06 RNASE3 PRTN3 PRG2 MPO COTL1
3 MAPK cascade GO:0000165 9.96 TNF IL5 IL3 IL2 IL1B
4 positive regulation of protein phosphorylation GO:0001934 9.95 TNF IL2 IL1B HLA-DRB1
5 response to lipopolysaccharide GO:0032496 9.93 THBD MPO IL1B IL10
6 cellular response to lipopolysaccharide GO:0071222 9.93 TNF IL1B IL10 CXCL8
7 defense response to bacterium GO:0042742 9.92 TNF RNASE3 PRG2 MPO IL10
8 defense response GO:0006952 9.91 TNF MPO CYSLTR1 CXCL8
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 9.89 TNF HLA-DRB1 CCL26 CCL17 CCL11
11 positive regulation of interleukin-6 production GO:0032755 9.86 TNF IL1B IL17A
12 cell-cell signaling GO:0007267 9.85 IL3 IL2 IL1B IL17A CCL26 CCL17
13 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.84 TNF IL3 IL2
14 positive regulation of DNA-binding transcription factor activity GO:0051091 9.84 TNF IL5 IL1B IL10
15 positive regulation of endothelial cell proliferation GO:0001938 9.83 IL10 CCL26 CCL11
16 positive regulation of interferon-gamma production GO:0032729 9.83 TNF IL2 IL1B
17 monocyte chemotaxis GO:0002548 9.79 CCL26 CCL17 CCL11
18 lymphocyte chemotaxis GO:0048247 9.77 CCL26 CCL17 CCL11
19 neutrophil chemotaxis GO:0030593 9.76 CXCL8 CCL26 CCL17 CCL11
20 inflammatory response GO:0006954 9.76 TNF IL5 IL1B IL17A CXCL8 CCL26
21 positive regulation of JAK-STAT cascade GO:0046427 9.74 TNF IL5 IL10
22 chemotaxis GO:0006935 9.73 RNASE3 CYSLTR1 CXCL8 CCL26 CCL17 CCL11
23 defense response to fungus GO:0050832 9.72 MPO IL17A COTL1
24 cellular response to interleukin-1 GO:0071347 9.72 IL17A CXCL8 CCL26 CCL17 CCL11
25 chemokine-mediated signaling pathway GO:0070098 9.71 CXCL8 CCL26 CCL17 CCL11
26 negative regulation of neurogenesis GO:0050768 9.69 TNF IL1B CCL11
27 positive regulation of neuroinflammatory response GO:0150078 9.68 TNF IL1B
28 positive regulation of podosome assembly GO:0071803 9.68 TNF IL5
29 response to molecule of bacterial origin GO:0002237 9.67 IL10 CXCL8
30 regulation of establishment of endothelial barrier GO:1903140 9.67 TNF IL1B
31 endothelial cell apoptotic process GO:0072577 9.65 TNF IL10
32 positive regulation of immunoglobulin production GO:0002639 9.65 IL5 IL2 IL10
33 cytokine-mediated signaling pathway GO:0019221 9.65 TNF PRTN3 IL5 IL3 IL2 IL1B
34 vascular endothelial growth factor production GO:0010573 9.64 TNF IL1B
35 negative regulation of cytokine production involved in immune response GO:0002719 9.64 TNF IL10
36 positive regulation of fever generation GO:0031622 9.62 TNF IL1B
37 sequestering of triglyceride GO:0030730 9.61 TNF IL1B
38 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.61 TNF IL1B IL10
39 positive regulation of plasma cell differentiation GO:1900100 9.58 IL2 IL10
40 positive regulation of calcidiol 1-monooxygenase activity GO:0060559 9.58 TNF IL1B
41 immune response GO:0006955 9.44 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 9.36 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
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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