COP
MCID: CRY001
MIFTS: 46

Cryptogenic Organizing Pneumonia (COP)

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Cryptogenic Organizing Pneumonia

MalaCards integrated aliases for Cryptogenic Organizing Pneumonia:

Name: Cryptogenic Organizing Pneumonia 12 53 59 44 15 17
Bronchiolitis Obliterans Organizing Pneumonia 12 53 59 72
Boop 12 53 59
Constrictive Bronchiolitis 53 72
Cop 53 59
Idiopathic Bronchiolitis Obliterans with Organizing Pneumonia 12
Idiopathic Bronchiolitis Obliterans Organizing Pneumonia 53
Cryptogenic Organizing Pneumonitis 12
Organizing Pneumonia 53
Organized Pneumonia 72
Idiopathic Boop 53

Characteristics:

Orphanet epidemiological data:

59
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: 59  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:0050157
ICD9CM 35 516.36
MeSH 44 D018549
NCIt 50 C62586
ICD10 33 J84.116
MESH via Orphanet 45 D018549
ICD10 via Orphanet 34 J84.1
UMLS via Orphanet 73 C0242770
Orphanet 59 ORPHA1302
UMLS 72 C0242770 C0264383 C2350875

Summaries for Cryptogenic Organizing Pneumonia

NIH Rare Diseases : 53 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.

MalaCards based summary : Cryptogenic Organizing Pneumonia, also known as bronchiolitis obliterans organizing pneumonia, is related to bronchiolitis obliterans and pneumothorax. An important gene associated with Cryptogenic Organizing Pneumonia is CXCL8 (C-X-C Motif Chemokine Ligand 8), and among its related pathways/superpathways are ERK Signaling and Cytokine Signaling in Immune system. The drugs Sotalol and Peripheral Nervous System Agents have been mentioned in the context of this disorder. Affiliated tissues include lung, breast and bone.

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 : 75 Cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans organizing pneumonia... 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 383)
# Related Disease Score Top Affiliating Genes
1 bronchiolitis obliterans 32.8 TIMP1 MMP9 CXCL8
2 pneumothorax 30.3 MUC1 MMP9
3 pulmonary disease, chronic obstructive 30.3 TIMP1 MMP9 CXCL8
4 pulmonary emphysema 30.2 TIMP1 MMP9
5 interstitial lung disease 30.0 TIMP1 MUC1 MMP9 CXCL8
6 pulmonary fibrosis, idiopathic 30.0 TIMP1 MUC1 MMP9 CXCL8
7 pneumocystosis 29.6 MUC1 CD4
8 bird fancier's lung 29.6 MUC1 CD8A
9 idiopathic interstitial pneumonia 11.8
10 pneumonia 11.0
11 lupus erythematosus 10.6
12 bronchiolitis 10.6
13 vibratory urticaria 10.5 MMP9 CXCL8
14 acute transverse myelitis 10.5 MMP9 CXCL8
15 transverse myelitis 10.5 MMP9 CXCL8
16 lung disease 10.5
17 periodontosis 10.5 MMP9 CXCL8
18 mycetoma 10.5 MMP9 CXCL8
19 tuberculous meningitis 10.4 MMP9 CXCL8
20 pyoderma 10.4 MMP9 CXCL8
21 respiratory failure 10.4
22 pulmonary fibrosis 10.4
23 autoimmune inner ear disease 10.4 TIMP1 MMP9
24 inner ear disease 10.4 TIMP1 MMP9
25 pneumothorax, primary spontaneous 10.4
26 common variable immunodeficiency 10.4
27 bronchopneumonia 10.4
28 fungal keratitis 10.4 TIMP1 MMP9
29 lung giant cell carcinoma 10.4 TIMP1 CXCL8
30 conjunctival nevus 10.4 TIMP1 MMP9
31 nasal cavity squamous cell carcinoma 10.3 TIMP1 MMP9
32 preterm premature rupture of the membranes 10.3 MMP9 CXCL8
33 pleural tuberculosis 10.3 TIMP1 CXCL8
34 maxillary cancer 10.3 TIMP1 HDAC9
35 light chain deposition disease 10.3 TNC MMP9
36 chronic venous insufficiency 10.3 TIMP1 MMP9
37 nonspecific interstitial pneumonia 10.3
38 connective tissue disease 10.3
39 gastroesophageal reflux 10.3
40 cystic fibrosis 10.3
41 human immunodeficiency virus type 1 10.3
42 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.3
43 pulmonary hypertension 10.3
44 lymphoma 10.3
45 lymphocytic leukemia 10.3
46 common cold 10.3
47 chlamydia 10.3
48 pancytopenia 10.3
49 systemic scleroderma 10.3
50 pulmonary coin lesion 10.3

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



Diseases related to Cryptogenic Organizing Pneumonia

Symptoms & Phenotypes for Cryptogenic Organizing Pneumonia

Drugs & Therapeutics for Cryptogenic Organizing Pneumonia

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

(show top 50) (show all 68)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sotalol Approved Phase 4 959-24-0, 3930-20-9 5253
2 Peripheral Nervous System Agents Phase 4
3 Autonomic Agents Phase 4
4 Neurotransmitter Agents Phase 4
5 Sodium Channel Blockers Phase 4
6 Diuretics, Potassium Sparing Phase 4
7 Adrenergic beta-Antagonists Phase 4
8 Adrenergic Agents Phase 4
9 Adrenergic Antagonists Phase 4
10 Anti-Arrhythmia Agents Phase 4
11 Sympatholytics Phase 4
12
Protein C Approved Phase 2
13
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
14
Sirolimus Approved, Investigational Phase 2 53123-88-9 6436030 5284616 46835353
15
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
16
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
17
Ribavirin Approved Phase 2 36791-04-5 37542
18
Oseltamivir Approved Phase 2 204255-11-8, 196618-13-0 65028
19
Montelukast Approved Phase 2 158966-92-8 5281040
20
Budesonide Approved Phase 2 51333-22-3 63006 5281004
21
Azithromycin Approved Phase 2 83905-01-5 447043 55185
22
Fluticasone Approved, Experimental Phase 2 90566-53-3 62924
23
Serine Approved, Nutraceutical Phase 2 56-45-1 5951
24 interferons Phase 2
25 Antiviral Agents Phase 2
26 Interferon-gamma Phase 2
27 Alpha 1-Antitrypsin Phase 2
28
protease inhibitors Phase 2
29 HIV Protease Inhibitors Phase 2
30 Serine Proteinase Inhibitors Phase 2
31 Trypsin Inhibitors Phase 2
32 Protein C Inhibitor Phase 2
33 Pharmaceutical Solutions Phase 2
34 Cyclosporins Phase 2
35 Calcineurin Inhibitors Phase 2
36 Antirheumatic Agents Phase 2
37 Antifungal Agents Phase 2
38 Anti-Infective Agents Phase 2
39 Immunosuppressive Agents Phase 2
40 Immunologic Factors Phase 2
41 Anti-Bacterial Agents Phase 2
42 Antibiotics, Antitubercular Phase 2
43 Immunoglobulins Phase 2
44 Antibodies Phase 2
45 glucocorticoids Phase 2
46 Respiratory System Agents Phase 2
47 Anti-Inflammatory Agents Phase 2
48 Hormones Phase 2
49 Cytochrome P-450 Enzyme Inducers Phase 2
50 Antineoplastic Agents, Hormonal Phase 2

Interventional clinical trials:

(show all 13)
# Name Status NCT ID Phase Drugs
1 A Pilot and Feasibility Study to Determine if a Common Atrial Fibrillation Risk Locus Modulates Differential Response to Antiarrhythmic Drugs Recruiting NCT02347111 Phase 4 Flecainide;Sotalol
2 Eine Phase II Studie über Interferon Gamma 1b Zur Behandlung Der steroidrefraktären Bronchiolitis Obliterans Nach Allogener SZT Unknown status NCT01639261 Phase 2 Interferon gamma 1b
3 Safety and Efficacy of Inhaled Alpha-1 Antitrypsin in Preventing Bronchiolitis Obliterable Syndrome in Lung Transplant Recipients Unknown status NCT01394835 Phase 2 Alpha -1 Antitrypsin;Alpha-1 Antitrypsin;AAT
4 A Dose-Ranging Study of the Efficacy and Safety of Bardoxolone Methyl in Patients With Pulmonary Hypertension Completed NCT02036970 Phase 2 Bardoxolone methyl;Placebo
5 Phase II Trial of Cyclosporine Inhalation Solution (CIS) in Lung Transplant and Hematopoietic Stem Cell Transplant Recipients for Treatment of Bronchiolitis Obliterans Completed NCT01287078 Phase 2 Cyclosporine Inhalation Solution
6 Treatment of Newly Diagnosed Moderate or Severe Chronic Graft-versus-host Disease With Prednisone and Everolimus (PredEver First) - A Prospective Multicenter Phase IIA Study - Active, not recruiting NCT01862965 Phase 2 PredEver
7 Does Increasing Immunosuppression Prevent Transplant-associated Lung-disease Triggered by Viral Respiratory Tract Infection Following Allogeneic Stem Cell Transplant? A Pilot Study Terminated NCT01432080 Phase 2 Prednisone;Azithromycin;Montelukast;Symbicort
8 Randomized Study of Early Treatment With Inhaled Corticosteroids Versus Observation for Patients Who Have Decreased Lung Function Status Post Allogeneic Stem Cell Transplantation Terminated NCT00656916 Phase 2 Fluticasone Propionate
9 Prospective, Phase I/II, Non-randomized, Open Label, Multicenter Study to Determine Safety and Efficacy of Nilotinib in a Population With Steroid-refractory/or Steroid-dependent cGVHD. Completed NCT01810718 Phase 1 nilotinib
10 Study of the Etiology and Immunological Pathogenesis in Acute Exacerbation of Idiopathic Pulmonary Fibrosis (AE-IPF) Unknown status NCT02509364
11 The European IPF Registry - an Internet-based, Pan-European Registry Linked to the European IPF Biobank (eurIPFbank) Recruiting NCT02951416
12 Evaluation of Regional Lung Function in U.S. Soldiers With Suspected Iraq-Afghanistan War Lung Injury Using 19F MRI Recruiting NCT03237364 Early Phase 1
13 Incidence, Clinical Characteristics and Evaluation of Prognostic and Diagnostic Markers of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study Not yet recruiting NCT03870828

Search NIH Clinical Center for Cryptogenic Organizing Pneumonia

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Prednisolone
Prednisone

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:

41
Lung, Breast, Bone, Bone Marrow, Liver, T Cells, Kidney

Publications for Cryptogenic Organizing Pneumonia

Articles related to Cryptogenic Organizing Pneumonia:

(show top 50) (show all 955)
# Title Authors PMID Year
1
Focal organizing pneumonia simulating lung malignancy: treated with prednisolone. 38
31406577 2019
2
Lung Segmentation on HRCT and Volumetric CT for Diffuse Interstitial Lung Disease Using Deep Convolutional Neural Networks. 38
31396776 2019
3
[Differences in small airway lesions in patients with different types of idiopathic interstitial pneumonia]. 38
31434411 2019
4
Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study. 38
31392498 2019
5
Squawks in interstitial lung disease prevalence and causes in a cohort of one thousand patients. 38
31335692 2019
6
Patterns of onset and outcome of cryptogenic organizing pneumonia after allogeneic hematopoietic stem cell transplantation. 38
30972616 2019
7
Organizing pneumonia co-existing with carcinoid tumour: complete resolution with bronchoscopic tumour resection. 38
30891244 2019
8
Characteristics and outcomes of patients hospitalized with interstitial lung diseases in Spain, 2014 to 2015. 38
31124970 2019
9
[The role of histone deacetylases in the pathogenesis of idiopathic pulmonary fibrosis and cryptogenic organizing pneumonia]. 38
31137112 2019
10
Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 1. Introduction. 38
31172699 2019
11
Impact and safety of open lung biopsy in patients with acute respiratory distress syndrome (ARDS). 38
29501285 2019
12
Nivolumab-induced interstitial lung disease in a patient with gastric cancer. 38
30863551 2019
13
Predictive factors for relapse of cryptogenic organizing pneumonia. 38
30626371 2019
14
Refractory Metastatic Insulinoma Treated with Everolimus, Complicated by Cryptogenic Organizing Pneumonia. 38
31414042 2019
15
Cryptogenic Organizing Pneumonia Presenting as a Solitary Mass: Clinical, Imaging, and Pathologic Features. 38
30648699 2019
16
[Post-transplant pulmonary complications: Guidelines from the francophone Society of bone marrow transplantation and cellular therapy (SFGM-TC)]. 38
30595221 2019
17
A long-term retrospective study of patients with biopsy-proven cryptogenic organizing pneumonia. 38
31159568 2019
18
Organizing pneumonia secondary to lung cancer of unknown primary site. 38
31312597 2019
19
Successful Management of Immune Thrombocytopenia Presenting with Lethal Alveolar Hemorrhage. 38
31281686 2019
20
Percutaneous computed tomography-guided localization of pulmonary nodules with hook wire prior to video-assisted thoracoscopic surgery. 38
30941979 2019
21
Elevated Levels of Intelectin-1, a Pathogen-binding Lectin, in the BAL Fluid of Patients with Chronic Eosinophilic Pneumonia and Hypersensitivity Pneumonitis. 38
30101907 2018
22
Bronchiolitis obliterans organizing pneumonia in Crohn's disease. 38
30578017 2018
23
Respiratory failure in the hematopoietic stem cell transplant recipient. 38
30370228 2018
24
Immune-checkpoint inhibitors in non-small cell lung cancer: A tool to improve patients' selection. 38
30097235 2018
25
Idiopathic interstitial pneumonias: review of the latest American Thoracic Society/European Respiratory Society classification. 38
30369660 2018
26
Transient Asymptomatic Pulmonary Opacities During Osimertinib Treatment and its Clinical Implication. 38
29775809 2018
27
Pulmonary infectious complications after hematopoietic stem cell transplantation: a practical guide to clinicians. 38
29889152 2018
28
Rituximab in the Treatment of Interstitial Lung Disease Associated with Antisynthetase Syndrome: A Multicenter Retrospective Case Review. 38
29606668 2018
29
Surgical lung biopsy in children after hematopoietic cell transplantation. 38
29602553 2018
30
Fibrotic lung toxicity induced by cytotoxic drugs, radiation and immunotherapy in patients treated for lung cancer. 38
29927195 2018
31
Oxaliplatin-induced bronchiolitis obliterans organizing pneumonia following hyperthermic intraperitoneal chemotherapy. 38
29998025 2018
32
Clinical Management of Pneumonitis in Patients Receiving Anti-PD-1/PD-L1 Therapy. 38
30719394 2018
33
Clinical characteristics of autoimmune rheumatic disease-related organizing pneumonia. 38
28748510 2018
34
Serial chest CT in cryptogenic organizing pneumonia: Evolutional changes and prognostic determinants. 38
28960643 2018
35
Bronchiolitis obliterans organizing pneumonia after stereotactic ablative radiation therapy for lung cancer: A case report. 38
29395853 2018
36
Cryptogenic organizing pneumonia masquerading as lung carcinoma: A case report and review of the literature. 38
29399056 2018
37
High incidence and early onset of nivolumab-induced pneumonitis: four case reports and literature review. 38
29378571 2018
38
Cryptogenic Organizing Pneumonia with Sarcoidosis Overlap: An Atypical Case Study. 38
30026760 2018
39
Distinct Profiles of CD163-Positive Macrophages in Idiopathic Interstitial Pneumonias. 38
29507864 2018
40
Noninfectious Acute Lung Injury Syndromes Early After Hematopoietic Stem Cell Transplantation. 38
29128012 2017
41
[Clinical features of acute diffuse pulmonary exudative disorders]. 38
29275576 2017
42
Concurrence of nivolumab-induced interstitial lung disease and cancer invasion. 38
28811904 2017
43
Development of bronchiolitis obliterans organizing pneumonia during standard treatment of hepatitis C with Peg-IFNα2b. 38
28823117 2017
44
Neuromyelitis optica spectrum disorder in the elderly associated with bronchiolitis obliterans organizing pneumonia. 38
29620080 2017
45
Urgent Lung Transplantation in Severe Acute Respiratory Failure Based on Rapidly Progressive Interstitial Lung Disease: A Case Report. 38
28838437 2017
46
Sex steroid receptor expression in idiopathic pulmonary fibrosis. 38
28300574 2017
47
[Medical prevention and treatment of radiation-induced pulmonary complications]. 38
28596060 2017
48
[Chinese expert consensus for non-antiinfective effects and clinical use of macrolides]. 38
28693067 2017
49
Exhaled nitric oxide measurements in patients with acute-onset interstitial lung disease. 38
28660859 2017
50
Early Onset Noninfectious Pulmonary Syndromes after Hematopoietic Cell Transplantation. 38
28477636 2017

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

Pathways related to Cryptogenic Organizing Pneumonia according to GeneCards Suite gene sharing:

(show all 16)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.5 TNC TIMP1 MUC1 LAMC2 CXCL8 CD4
2
Show member pathways
12.96 TIMP1 MUC1 MMP9 CXCL8 CD4
3
Show member pathways
12.73 TNC TIMP1 LAMC2 HDAC9
4
Show member pathways
12.32 TNC TIMP1 MMP9 LAMC2
5
Show member pathways
11.84 TNC MMP9 LAMC2 CXCL8
6 11.45 TNC TIMP1 MMP9
7 11.31 TIMP1 MUC1 MMP9 CXCL8
8 11.24 TIMP1 CXCL8
9 11.23 TIMP1 MMP9 CXCL8
10 11.09 CD8A CD4
11 11.04 MMP9 CXCL8
12 10.99 TNC MMP9
13 10.92 TNC MUC1 MMP9
14 10.88 TIMP1 MMP9
15 10.71 TIMP1 MMP9 LAMC2 CXCL8
16 10.61 TNC TIMP1 LAMC2 CXCL8

GO Terms for Cryptogenic Organizing Pneumonia

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.73 TNC TIMP1 MUC1 MMP9 LAMC2 CXCL8
2 endoplasmic reticulum lumen GO:0005788 9.58 TNC TIMP1 CD4
3 collagen-containing extracellular matrix GO:0062023 9.54 TNC MMP9 LAMC2
4 basement membrane GO:0005604 9.33 TNC TIMP1 LAMC2
5 T cell receptor complex GO:0042101 9.32 CD8A CD4
6 extracellular matrix GO:0031012 9.26 TNC TIMP1 MMP9 LAMC2
7 extracellular region GO:0005576 9.17 TNC TIMP1 MUC1 MMP9 LAMC2 CXCL8

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

# Name GO ID Score Top Affiliating Genes
1 extracellular matrix disassembly GO:0022617 9.26 TIMP1 MMP9
2 T cell activation GO:0042110 9.16 CD8A CD4
3 extracellular matrix organization GO:0030198 9.13 TNC MMP9 LAMC2
4 cytokine-mediated signaling pathway GO:0019221 9.02 TIMP1 MUC1 MMP9 CXCL8 CD4

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

# Name GO ID Score Top Affiliating Genes
1 coreceptor activity GO:0015026 8.96 CD8A CD4
2 extracellular matrix structural constituent GO:0005201 8.8 TNC LAMC2 CD4

Sources for Cryptogenic Organizing Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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