COP
MCID: CRY001
MIFTS: 44

Cryptogenic Organizing Pneumonia (COP) malady

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Cryptogenic Organizing Pneumonia

Aliases & Descriptions for Cryptogenic Organizing Pneumonia:

Name: Cryptogenic Organizing Pneumonia 12 50 56 42 14
Bronchiolitis Obliterans Organizing Pneumonia 12 50 56 69
Constrictive Bronchiolitis 50 69
Boop 12 50
Cop 50 56
Idiopathic Bronchiolitis Obliterans with Organizing Pneumonia 12
Idiopathic Bronchiolitis Obliterans Organizing Pneumonia 50
Cryptogenic Organizing Pneumonitis 12
Organizing Pneumonia 50
Organized Pneumonia 69
Idiopathic Boop 50

Characteristics:

Orphanet epidemiological data:

56
cryptogenic organizing pneumonia
Inheritance: Not applicable; Prevalence: 1-9/100000 (Iceland); Age of onset: Adolescent,Adult,Elderly; Age of death: normal life expectancy;

Classifications:

Orphanet: 56  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:0050157
ICD10 33 J84.116
ICD9CM 35 516.36
MeSH 42 D018549
NCIt 47 C62586
SNOMED-CT 64 129458007
Orphanet 56 ORPHA1302
MESH via Orphanet 43 D018549
ICD10 via Orphanet 34 J84.1
UMLS 69 C0242770

Summaries for Cryptogenic Organizing Pneumonia

NIH Rare Diseases : 50 bronchiolitis obliterans organizing pneumonia (boop) is a lung disease that causes inflammation in the small air tubes (bronchioles) and air sacs (alveoli). boop typically develops in individuals between 40-60 years old; however the disorder may affect individuals of any age. the signs and symptoms of boop vary but often include shortness of breath, a dry cough, and fever. boop can be caused by viral infections, various drugs, and other medical conditions. if the cause is known, the condition is called secondary boop. in many cases, the underlying cause of boop is unknown. these cases are called idiopathic boop or cryptogenic organizing pneumonia. treatment often includes corticosteroid medications. last updated: 2/16/2016

MalaCards based summary : Cryptogenic Organizing Pneumonia, also known as bronchiolitis obliterans organizing pneumonia, is related to idiopathic interstitial pneumonia and bronchiolitis obliterans with obstructive pulmonary disease. An important gene associated with Cryptogenic Organizing Pneumonia is CXCL8 (C-X-C Motif Chemokine Ligand 8), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Bosentan and Antihypertensive Agents have been mentioned in the context of this disorder. Affiliated tissues include lung, breast and neutrophil, and related phenotype is Decreased HIV-LTR-beta-galactosidase protein expression.

Disease Ontology : 12 An idiopathic interstitial pneumonia characterized by lung inflammation and scarring that obstructs the small airways and air sacs of the lungs (alveoli). A flu-like illness, with a cough, fever, a feeling of illness (malaise), fatigue, and weight loss, heralds the onset in about 50% of people. Frequent presence of crackling sounds (called Velcro crackles) when the doctor listens with a stethoscope.

Wikipedia : 71 Bronchiolitis obliterans organizing pneumonia (BOOP), also known as cryptogenic organizing pneumonia, is... more...

Related Diseases for Cryptogenic Organizing Pneumonia

Diseases related to Cryptogenic Organizing Pneumonia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 121)
id Related Disease Score Top Affiliating Genes
1 idiopathic interstitial pneumonia 11.1
2 bronchiolitis obliterans with obstructive pulmonary disease 10.8
3 pneumonia 10.7
4 hypersensitivity reaction type iii disease 10.3 CXCL8 MMP9
5 neonatal hypoxic and ischemic brain injury 10.3 CXCL8 MMP9
6 congenital hypomyelination neuropathy 10.3 MMP9 TIMP1
7 chondrodysplasia punctata, rhizomelic, type 1 10.3 MMP9 TIMP1
8 appendix carcinoid tumor 10.3 MMP9 TIMP1
9 schwannomatosis 10.2 MMP9 TIMP1
10 omsk hemorrhagic fever 10.2 CXCL8 MMP9
11 striated muscle rhabdoid tumor 10.2 MMP9 TIMP1
12 trichomalacia 10.2 CXCL8 MMP9
13 adenoid squamous cell carcinoma 10.2 MMP9 TIMP1
14 glucose intolerance 10.2 CXCL8 TIMP1
15 linear porokeratosis 10.2 MMP9 TNC
16 retinal drusen 10.2 MMP9 TIMP1
17 optic atrophy 2 10.2 MUC5B TNC
18 diversion colitis 10.2 CD8A MMP9
19 histiocytoma 10.2 CXCL8 MMP9
20 breast papillary carcinoma 10.2 CXCL8 MMP9 TIMP1
21 chordoid meningioma 10.1 CXCL8 MMP9 TIMP1
22 burns 10.1 CXCL8 MMP9 TIMP1
23 small intestine lymphoma 10.1 CXCL8 MUC5B
24 histiocytosis 10.1 CXCL8 MMP9 TIMP1
25 intratubular embryonal carcinoma 10.1 CXCL8 MMP9 TIMP1
26 laryngeal mucoepidermoid carcinoma 10.1 MUC1 MUC5B
27 fibular collateral ligament bursitis 10.1 MUC1 MUC5B
28 bronchiolitis 10.1
29 in situ pulmonary adenocarcinoma 10.1 CXCL8 MMP9 TNC
30 refractory plasma cell neoplasm 10.1 MUC1 MUC5B
31 maxillary neoplasm 10.1 MMP9 TIMP1 TNC
32 autoimmune disease of gastrointestinal tract 10.1 CD8A MUC5B
33 total internal ophthalmoplegia 10.1 CD4 CXCL8
34 brachydactyly, type b1 10.1 MUC1 MUC5B
35 contact lens corneal edema 10.1 CXCL8 TNC
36 bronchiolitis obliterans 10.1
37 postinflammatory pulmonary fibrosis 10.1 MUC1 MUC5B
38 retina lymphoma 10.1 MUC1 MUC5B
39 bullous retinoschisis 10.1 CD4 CXCL8
40 carbuncle 10.1 CD4 CXCL8
41 prostate embryonal rhabdomyosarcoma 10.0 MUC1 MUC5B
42 lung clear cell carcinoma 10.0 MUC1 MUC5B
43 sclerosing cholangitis 10.0 CD4 CXCL8
44 dermatitis herpetiformis 10.0 CD4 MUC1
45 orthostatic proteinuria 10.0 CXCL8 MUC1
46 omodysplasia 2 10.0 CD4 MUC1
47 alcoholic pancreatitis 10.0 CD4 CD8A
48 orbital disease 10.0 CD4 CD8A
49 colloid adenoma 10.0 CD4 CD8A
50 retinal microaneurysm 10.0 CD4 CD8A

Graphical network of the top 20 diseases related to Cryptogenic Organizing Pneumonia:



Diseases related to Cryptogenic Organizing Pneumonia

Symptoms & Phenotypes for Cryptogenic Organizing Pneumonia

GenomeRNAi Phenotypes related to Cryptogenic Organizing Pneumonia according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased HIV-LTR-beta-galactosidase protein expression GR00224-A-1 8.8 CD4 MMP9 MUC5B

Drugs & Therapeutics for Cryptogenic Organizing Pneumonia

Drugs for Cryptogenic Organizing Pneumonia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 57)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 3 147536-97-8 104865
2 Antihypertensive Agents Phase 3
3 Endothelin Receptor Antagonists Phase 3
4
Azithromycin Approved Phase 2 83905-01-5 55185 53477736 447043
5
Budesonide Approved Phase 2 51333-22-3 63006 5281004
6
Montelukast Approved Phase 2 158966-92-8 5281040
7
Oseltamivir Approved Phase 2 204255-11-8, 196618-13-0 65028
8
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
9
Ribavirin Approved Phase 2 36791-04-5 37542
10
Pirfenidone Investigational Phase 2 53179-13-8 40632
11 Alpha 1-Antitrypsin Phase 2
12 HIV Protease Inhibitors Phase 2
13
protease inhibitors Phase 2
14 Protein C Phase 2
15 Protein C Inhibitor Phase 2
16 Serine Proteinase Inhibitors Phase 2
17 Trypsin Inhibitors Phase 2
18 Analgesics Phase 2
19 Analgesics, Non-Narcotic Phase 2
20 Anti-Inflammatory Agents Phase 2
21 Anti-Inflammatory Agents, Non-Steroidal Phase 2
22 Antirheumatic Agents Phase 2
23 Peripheral Nervous System Agents Phase 2,Phase 1
24 Phosphodiesterase 5 Inhibitors Phase 2
25 Phosphodiesterase Inhibitors Phase 2
26 Sildenafil Citrate Phase 2 171599-83-0
27 Vasodilator Agents Phase 2,Phase 1
28 Antibodies Phase 2
29 Immunoglobulins Phase 2
30 Anti-Asthmatic Agents Phase 2,Phase 1
31 Respiratory System Agents Phase 2,Phase 1
32 Autonomic Agents Phase 2,Phase 1
33 Bronchodilator Agents Phase 2,Phase 1
34 Antineoplastic Agents, Hormonal Phase 2
35 Antipyretics Phase 2
36 Budesonide, Formoterol Fumarate Drug Combination Phase 2
37 Formoterol Fumarate Phase 2
38 gamma-Globulins Phase 2
39 glucocorticoids Phase 2
40 Hormone Antagonists Phase 2
41 Hormones Phase 2
42 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
43 Immunoglobulins, Intravenous Phase 2
44 Leukotriene Antagonists Phase 2
45 Rho(D) Immune Globulin Phase 2
46 Anti-Allergic Agents Phase 2
47 Dermatologic Agents Phase 2
48
Fluticasone Phase 2 90566-53-3, 80474-14-2 62924 22833648
49 serine Nutraceutical Phase 2
50 Citrate Nutraceutical Phase 2

Interventional clinical trials:

(show all 11)
id Name Status NCT ID Phase
1 BUILD 3: Bosentan Use in Interstitial Lung Disease Completed NCT00391443 Phase 3
2 Safety and Efficacy of Inhaled Alpha-1 Antitrypsin in Preventing Bronchiolitis Obliterable Syndrome in Lung Transplant Recipients Unknown status NCT01394835 Phase 2
3 Efficacy, Safety, and Tolerability Study of Pirfenidone in Combination With Sildenafil in Participants With Advanced Idiopathic Pulmonary Fibrosis (IPF) and Intermediate or High Probability of Group 3 Pulmonary Hypertension Recruiting NCT02951429 Phase 2
4 Steroids, Azithromycin, Montelukast, and Symbicort (SAMS) for Viral Respiratory Tract Infection Post Allotransplant Suspended NCT01432080 Phase 2
5 Inhaled Corticosteroids Versus Observation for Patients With Decreased Lung Function Status Terminated NCT00656916 Phase 2
6 3 Part Study to Assess Inhaled Nitric Oxide on Functional Pulmonary Imaging in Subjects Pulmonary Hypertension Associated With COPD and IPF Active, not recruiting NCT02267655 Phase 1
7 The Occurence of Inflammation and Oxidative Stress in Lung Diseases Completed NCT00512967
8 Prospective Study of Belated Pulmonary Complications Occurring in Children Treated With Allogeneic Hematopoietic Stem Cells. Recruiting NCT02032381
9 Lung MRI in the Management of Idiopathic Pulmonary Fibrosis Recruiting NCT03078426
10 Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank Recruiting NCT02951416
11 Japanese Idiopathic Interstitial Pneumonias Registry Recruiting NCT03041623

Search NIH Clinical Center for Cryptogenic Organizing Pneumonia

Cochrane evidence based reviews: cryptogenic organizing pneumonia

Genetic Tests for Cryptogenic Organizing Pneumonia

Anatomical Context for Cryptogenic Organizing Pneumonia

MalaCards organs/tissues related to Cryptogenic Organizing Pneumonia:

39
Lung, Breast, Neutrophil, Kidney, Colon

Publications for Cryptogenic Organizing Pneumonia

Articles related to Cryptogenic Organizing Pneumonia:

(show top 50) (show all 87)
id Title Authors Year
1
Radial Probe Endobronchial Ultrasound-guided Lung Biopsy for Histopathological Diagnosis of Cryptogenic Organizing Pneumonia. ( 28091418 )
2017
2
Clarithromycin Decreases IL-6 Concentration in Serum and BAL Fluid in Patients with Cryptogenic Organizing Pneumonia. ( 28028949 )
2016
3
Erythema gyratum repens associated with cryptogenic organizing pneumonia. ( 26765132 )
2016
4
Cryptogenic Organizing Pneumonia: IL-1I^, IL-6, IL-8, and TGF- I^1 Serum Concentrations and Response to Clarithromycin Treatment. ( 26987326 )
2016
5
Spontaneous resolution of cryptogenic organizing pneumonia in pediatrics: A case report. ( 27021808 )
2016
6
Azacitidine-induced cryptogenic organizing pneumonia: a case report and review of the literature. ( 26791087 )
2016
7
Cryptogenic organizing pneumonia: clinical and radiological features, treatment outcomes of 17 patients, and review of the literature. ( 28081314 )
2016
8
Macrolide therapy in cryptogenic organizing pneumonia: A case report and literature review. ( 25667636 )
2015
9
A Case of Cryptogenic Organizing Pneumonia after Transarterial Chemoembolization for the Treatment of Hepatocellular Carcinoma. ( 26508948 )
2015
10
Reversed Halo Sign. A Case of Cryptogenic Organizing Pneumonia with Spontaneous Resolution. ( 26131991 )
2015
11
Different Responses to Clarithromycin in Patients with Cryptogenic Organizing Pneumonia. ( 26508933 )
2015
12
Increased Galectin-9 Concentration and Number of CD4+Foxp3high+Cells in Bronchoalveolar Lavage Fluid of Patients with Cryptogenic Organizing Pneumonia. ( 26249221 )
2015
13
Haemoptysis as a primary manifestation of cryptogenic organizing pneumonia (COP). ( 27451594 )
2015
14
Successful Rituximab Therapy in Steroid-Resistant, Cryptogenic Organizing Pneumonia: A Case Series. ( 26045243 )
2015
15
Cryptogenic organizing pneumonia associated with invasive pulmonary aspergillosis: a case report and review of the literature. ( 25674228 )
2014
16
Cryptogenic organizing pneumonia associated with radiation: A report of two cases. ( 24396439 )
2014
17
Association of cryptogenic organizing pneumonia in bilateral anterior uveitis. ( 25520652 )
2014
18
The spectrum of presentations of cryptogenic organizing pneumonia in high resolution computed tomography. ( 25493105 )
2014
19
Macrolide use leads to clinical and radiological improvement in patients with cryptogenic organizing pneumonia. ( 24460438 )
2014
20
Bronchoalveolar lavage fluid and blood natural killer and natural killer T-like cells in cryptogenic organizing pneumonia. ( 24889556 )
2014
21
Fatal case of cryptogenic organizing pneumonia associated with Everolimus. ( 25827702 )
2014
22
Cryptogenic organizing pneumonia in Sweet's syndrome: case report and review of the literature. ( 25196175 )
2014
23
Cryptogenic organizing pneumonia during adjuvant chemotherapy with oxaliplatin, 5-fluorouracil, and leucovorin (FOLFOX) for colon cancer. ( 22842502 )
2013
24
Clinicopathologic features associated with relapse in cryptogenic organizing pneumonia. ( 24342432 )
2013
25
Pre-transplant risk factors for cryptogenic organizing pneumonia/bronchiolitis obliterans organizing pneumonia after hematopoietic cell transplantation. ( 23933758 )
2013
26
Cryptogenic organizing pneumonia in Tomm5(-/-) mice. ( 22688586 )
2013
27
Autopsy findings of fatal cryptogenic organizing pneumonia. ( 23696931 )
2013
28
Cryptogenic organizing pneumonia: A rare extra-intestinal manifestation of Crohn's disease. ( 24090908 )
2013
29
Simultaneous diagnosis of cryptogenic organizing pneumonia and HIV in a 45 year old man. ( 23569519 )
2012
30
Rituximab-related cryptogenic organizing pneumonia and late onset neutropenia in a patient with non-Hodgkin lymphoma: report of two rare complications and review of the literature. ( 23033310 )
2012
31
Cryptogenic organizing pneumonia with fatal respiratory failure. ( 22372175 )
2012
32
Cryptogenic organizing pneumonia. ( 23001801 )
2012
33
Chronic exposure to particles caused bronchioloalveolar carcinoma in a patient with cryptogenic organizing pneumonia evaluated by elemental analysis. ( 21675931 )
2012
34
Optimizing the utility of high-resolution computed tomography in diagnosing cryptogenic organizing pneumonia. ( 21093240 )
2011
35
Comparison between cryptogenic organizing pneumonia and connective tissue disease-related organizing pneumonia. ( 21169342 )
2011
36
A case of adult onset Still's disease complicated with cryptogenic organizing pneumonia. ( 21297329 )
2011
37
Increased expression of tumor necrosis factor receptors in cryptogenic organizing pneumonia. ( 21144722 )
2011
38
Cryptogenic organizing pneumonia mimicking malignancy in a patient with rheumatoid arthritis. ( 21885507 )
2011
39
Reversed halo sign in active pulmonary tuberculosis: criteria for differentiation from cryptogenic organizing pneumonia. ( 22109285 )
2011
40
Adjunctive effects of cyclosporine and macrolide in rapidly progressive cryptogenic organizing pneumonia with no prompt response to steroid. ( 21372463 )
2011
41
Utility of high-resolution computed tomography and BAL in cryptogenic organizing pneumonia. ( 20594817 )
2010
42
Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. ( 20858818 )
2010
43
Signal recognition particle (SRP) positive myositis in a patient with cryptogenic organizing pneumonia (COP). ( 19639324 )
2010
44
Kikuchi Fujimoto disease associated with cryptogenic organizing pneumonia: case report and literature review. ( 20222953 )
2010
45
Cryptogenic organizing pneumonia due to amiodarone: long-term follow-up after corticosteroid treatment. ( 20668958 )
2010
46
Re: Reversed halo sign: nodular wall as criterion for differentiation between cryptogenic organizing pneumonia and active granulomatous diseases. ( 20696310 )
2010
47
Cryptogenic organizing pneumonia after radiotherapy for breast cancer. ( 22958364 )
2010
48
Cryptogenic organizing pneumonia after rituximab therapy for presumed post-kidney transplant lymphoproliferative disease. ( 20140460 )
2010
49
Elevated levels of thioredoxin 1 in the lungs and sera of idiopathic pulmonary fibrosis, non-specific interstitial pneumonia and cryptogenic organizing pneumonia. ( 21088339 )
2010
50
Clinical and radiologic distinctions between secondary bronchiolitis obliterans organizing pneumonia and cryptogenic organizing pneumonia. ( 19650943 )
2009

Variations for Cryptogenic Organizing Pneumonia

Expression for Cryptogenic Organizing Pneumonia

Search GEO for disease gene expression data for Cryptogenic Organizing Pneumonia.

Pathways for Cryptogenic Organizing Pneumonia

GO Terms for Cryptogenic Organizing Pneumonia

Cellular components related to Cryptogenic Organizing Pneumonia according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 proteinaceous extracellular matrix GO:0005578 9.56 LAMC2 MMP9 TIMP1 TNC
2 endoplasmic reticulum lumen GO:0005788 9.54 CD4 TIMP1 TNC
3 extracellular space GO:0005615 9.5 CXCL8 LAMC2 MMP9 MUC1 MUC5B TIMP1
4 basement membrane GO:0005604 9.33 LAMC2 TIMP1 TNC
5 T cell receptor complex GO:0042101 9.32 CD4 CD8A
6 extracellular region GO:0005576 9.23 CD8A CXCL8 LAMC2 MMP9 MUC1 MUC5B

Biological processes related to Cryptogenic Organizing Pneumonia according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 extracellular matrix organization GO:0030198 9.33 LAMC2 MMP9 TNC
2 positive regulation of calcium-mediated signaling GO:0050850 8.96 CD4 CD8A
3 extracellular matrix disassembly GO:0022617 8.8 LAMC2 MMP9 TIMP1

Molecular functions related to Cryptogenic Organizing Pneumonia according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 coreceptor activity GO:0015026 8.62 CD4 CD8A

Sources for Cryptogenic Organizing Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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