COP
MCID: CRY001
MIFTS: 47

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 54 60 45 15 17
Bronchiolitis Obliterans Organizing Pneumonia 12 54 60 74
Boop 12 54 60
Constrictive Bronchiolitis 54 74
Cop 54 60
Idiopathic Bronchiolitis Obliterans with Organizing Pneumonia 12
Idiopathic Bronchiolitis Obliterans Organizing Pneumonia 54
Cryptogenic Organizing Pneumonitis 12
Organizing Pneumonia 54
Organized Pneumonia 74
Idiopathic Boop 54

Characteristics:

Orphanet epidemiological data:

60
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: 60  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:0050157
ICD9CM 36 516.36
MeSH 45 D018549
NCIt 51 C62586
ICD10 34 J84.116
MESH via Orphanet 46 D018549
ICD10 via Orphanet 35 J84.1
UMLS via Orphanet 75 C0242770
Orphanet 60 ORPHA1302

Summaries for Cryptogenic Organizing Pneumonia

NIH Rare Diseases : 54 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 Degradation of the extracellular matrix and G-protein signaling Regulation of p38 and JNK signaling mediated by G-proteins. The drugs Protein C and Azithromycin have been mentioned in the context of this disorder. Affiliated tissues include lung, breast and kidney.

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 : 77 Cryptogenic organizing pneumonia (COP), also 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 216)
# Related Disease Score Top Affiliating Genes
1 bronchiolitis obliterans 32.6 CXCL8 MMP9 TIMP1
2 pneumothorax 30.6 MMP9 MUC1
3 inguinal hernia 29.9 MMP9 TIMP1
4 pulmonary fibrosis, idiopathic 29.8 CXCL8 MMP9 MUC1 TIMP1
5 bird fancier's lung 29.5 CD8A MUC1
6 idiopathic interstitial pneumonia 11.4
7 pneumonia 10.9
8 systemic lupus erythematosus 10.6
9 lupus erythematosus 10.6
10 bronchiolitis 10.3
11 vibratory urticaria 10.3 CXCL8 MMP9
12 acute transverse myelitis 10.3 CXCL8 MMP9
13 transverse myelitis 10.3 CXCL8 MMP9
14 autoimmune inner ear disease 10.3 MMP9 TIMP1
15 periodontosis 10.3 CXCL8 MMP9
16 inner ear disease 10.3 MMP9 TIMP1
17 mycetoma 10.3 CXCL8 MMP9
18 tuberculous meningitis 10.3 CXCL8 MMP9
19 fungal keratitis 10.3 MMP9 TIMP1
20 conjunctival nevus 10.3 MMP9 TIMP1
21 lung giant cell carcinoma 10.3 CXCL8 TIMP1
22 pyoderma 10.3 CXCL8 MMP9
23 pleural tuberculosis 10.3 CXCL8 TIMP1
24 leukemia, chronic myeloid 10.3
25 hepatitis 10.3
26 lymphocytic leukemia 10.3
27 hepatitis c 10.3
28 eosinophilic pneumonia 10.3
29 chronic eosinophilic pneumonia 10.3
30 acute respiratory distress syndrome 10.3
31 chronic graft versus host disease 10.3
32 nasal cavity squamous cell carcinoma 10.3 MMP9 TIMP1
33 chronic venous insufficiency 10.2 MMP9 TIMP1
34 venous insufficiency 10.2 MMP9 TIMP1
35 preterm premature rupture of the membranes 10.2 CXCL8 MMP9
36 breast cancer 10.2
37 bronchopulmonary dysplasia 10.2 CXCL8 TIMP1
38 central nervous system tuberculosis 10.2 MMP9 TIMP1
39 light chain deposition disease 10.2 MMP9 TNC
40 aortic aneurysm, familial abdominal, 1 10.2 MMP9 TIMP1
41 toxic myocarditis 10.2 CXCL8 PIK3C2A
42 status asthmaticus 10.2 CXCL8 PIK3C2A
43 bullous keratopathy 10.2 CXCL8 TNC
44 pulmonary emphysema 10.2 MMP9 TIMP1
45 adult respiratory distress syndrome 10.2
46 spinal cord injury 10.2 CXCL8 MMP9
47 carotid artery disease 10.2 MMP9 TIMP1
48 chorioamnionitis 10.2 CXCL8 MMP9
49 fasciitis 10.2 CXCL8 MUC1
50 gingivitis 10.1 CXCL8 MMP9

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 51)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Protein C Approved Phase 2
2
Azithromycin Approved Phase 2 83905-01-5 55185 447043
3
Budesonide Approved Phase 2 51333-22-3 63006 5281004
4
Oseltamivir Approved Phase 2 204255-11-8, 196618-13-0 65028
5
Ribavirin Approved Phase 2 36791-04-5 37542
6
Montelukast Approved Phase 2 158966-92-8 5281040
7
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
8
Fluticasone Approved, Experimental Phase 2 90566-53-3 62924
9
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
10
Serine Approved, Nutraceutical Phase 2 56-45-1 5951
11 HIV Protease Inhibitors Phase 2
12
protease inhibitors Phase 2
13 Protein C Inhibitor Phase 2
14 Trypsin Inhibitors Phase 2
15 Alpha 1-Antitrypsin Phase 2
16 Serine Proteinase Inhibitors Phase 2
17 Budesonide, Formoterol Fumarate Drug Combination Phase 2
18 glucocorticoids Phase 2
19 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
20 Leukotriene Antagonists Phase 2
21 Antibodies Phase 2
22 Antineoplastic Agents, Hormonal Phase 2
23 Hormone Antagonists Phase 2
24 Respiratory System Agents Phase 2
25 Antipyretics Phase 2
26 Formoterol Fumarate Phase 2
27 Immunoglobulins, Intravenous Phase 2
28 gamma-Globulins Phase 2
29 Hormones Phase 2
30 Anti-Asthmatic Agents Phase 2
31 Anti-Inflammatory Agents Phase 2
32 Rho(D) Immune Globulin Phase 2
33 Immunoglobulins Phase 2
34 Anti-Allergic Agents Phase 2
35 Peripheral Nervous System Agents Phase 2
36 Autonomic Agents Phase 2
37 Bronchodilator Agents Phase 2
38 Dermatologic Agents Phase 2
39 Antifungal Agents Phase 2
40 Cyclosporins Phase 2
41 Calcineurin Inhibitors Phase 2
42 Pharmaceutical Solutions Phase 2
43 Antirheumatic Agents Phase 2
44 Immunologic Factors Phase 2
45 Immunosuppressive Agents Phase 2
46 Anti-Infective Agents Phase 2
47
Midazolam Approved, Illicit 59467-70-8 4192
48
Lidocaine Approved, Vet_approved 137-58-6 3676
49 Anesthetics
50 Calamus

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 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
2 Steroids, Azithromycin, Montelukast, and Symbicort (SAMS) for Viral Respiratory Tract Infection Post Allotransplant Terminated NCT01432080 Phase 2 Prednisone;Azithromycin;Montelukast;Symbicort
3 Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT Completed NCT02036970 Phase 2 Bardoxolone methyl;Placebo
4 Inhaled Corticosteroids Versus Observation for Patients With Decreased Lung Function Status Terminated NCT00656916 Phase 2 Fluticasone Propionate
5 Cyclosporine Inhalation Solution (CIS) in Lung Transplant and Hematopoietic Stem Cell Transplant Recipients for the Treatment of Bronchiolitis Obliterans Completed NCT01287078 Phase 2 Cyclosporine Inhalation Solution
6 Lung MRI in the Management of Idiopathic Pulmonary Fibrosis Unknown status NCT03078426 Not Applicable
7 Clinical Characteristics of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study Not yet recruiting NCT03870828
8 Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank Recruiting NCT02951416
9 Iraq- Afghanistan War Lung Injury Using 19F MRI (DIAL1001006) Recruiting NCT03237364 Early Phase 1
10 Prospective Study of Belated Pulmonary Complications Occurring in Children Treated With Allogeneic Hematopoietic Stem Cells. Active, not recruiting NCT02032381 Not Applicable

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:

42
Lung, Breast, Kidney, Colon, Neutrophil

Publications for Cryptogenic Organizing Pneumonia

Articles related to Cryptogenic Organizing Pneumonia:

(show top 50) (show all 111)
# Title Authors Year
1
Predictive factors for relapse of cryptogenic organizing pneumonia. ( 30626371 )
2019
2
Cryptogenic Organizing Pneumonia Presenting as a Solitary Mass: Clinical, Imaging, and Pathologic Features. ( 30648699 )
2019
3
Serial chest CT in cryptogenic organizing pneumonia: Evolutional changes and prognostic determinants. ( 28960643 )
2018
4
Cryptogenic organizing pneumonia masquerading as lung carcinoma: A case report and review of the literature. ( 29399056 )
2018
5
Bronchiolitis obliterans organizing pneumonia in Crohn's disease. ( 30578017 )
2018
6
Oxaliplatin-induced bronchiolitis obliterans organizing pneumonia following hyperthermic intraperitoneal chemotherapy. ( 29998025 )
2018
7
Cryptogenic Organizing Pneumonia with Sarcoidosis Overlap: An Atypical Case Study. ( 30026760 )
2018
8
Nocardia brasiliensis Infection Complicating Cryptogenic Organizing Pneumonia. ( 28348912 )
2017
9
Cryptogenic Organizing Pneumonia With Lung Nodules Secondary to Pulmonary Manifestation of Crohn Disease. ( 28579861 )
2017
10
Cicatricial variant of cryptogenic organizing pneumonia. ( 28412206 )
2017
11
Cryptogenic organizing pneumonia-Results of treatment with clarithromycin versus corticosteroids-Observational study. ( 28945804 )
2017
12
Retrospective evaluation of patients with organizing pneumonia: is cryptogenic organizing pneumonia different from secondary organizing pneumonia? ( 28621243 )
2017
13
Radial Probe Endobronchial Ultrasound-guided Lung Biopsy for Histopathological Diagnosis of Cryptogenic Organizing Pneumonia. ( 28091418 )
2017
14
Lung injury pathways: Adenosine receptor 2B signaling limits development of ischemic bronchiolitis obliterans organizing pneumonia. ( 28266889 )
2017
15
Extracorporeal membrane oxygenation rescue in adolescent with bronchiolitis obliterans-organizing pneumonia like Wegener's granulomatosis. ( 28096986 )
2017
16
Azacitidine-induced cryptogenic organizing pneumonia: a case report and review of the literature. ( 26791087 )
2016
17
Cryptogenic organizing pneumonia: clinical and radiological features, treatment outcomes of 17 patients, and review of the literature. ( 28081314 )
2016
18
Clarithromycin Decreases IL-6 Concentration in Serum and BAL Fluid in Patients with Cryptogenic Organizing Pneumonia. ( 28028949 )
2016
19
Erythema gyratum repens associated with cryptogenic organizing pneumonia. ( 26765132 )
2016
20
Spontaneous resolution of cryptogenic organizing pneumonia in pediatrics: A case report. ( 27021808 )
2016
21
Cryptogenic Organizing Pneumonia: IL-1I^, IL-6, IL-8, and TGF- I^1 Serum Concentrations and Response to Clarithromycin Treatment. ( 26987326 )
2016
22
Macrolide therapy in cryptogenic organizing pneumonia: A case report and literature review. ( 25667636 )
2015
23
Haemoptysis as a primary manifestation of cryptogenic organizing pneumonia (COP). ( 27451594 )
2015
24
Reversed Halo Sign. A Case of Cryptogenic Organizing Pneumonia with Spontaneous Resolution. ( 26131991 )
2015
25
Successful Rituximab Therapy in Steroid-Resistant, Cryptogenic Organizing Pneumonia: A Case Series. ( 26045243 )
2015
26
A Case of Cryptogenic Organizing Pneumonia after Transarterial Chemoembolization for the Treatment of Hepatocellular Carcinoma. ( 26508948 )
2015
27
Different Responses to Clarithromycin in Patients with Cryptogenic Organizing Pneumonia. ( 26508933 )
2015
28
Increased Galectin-9 Concentration and Number of CD4+Foxp3high+Cells in Bronchoalveolar Lavage Fluid of Patients with Cryptogenic Organizing Pneumonia. ( 26249221 )
2015
29
Cryptogenic organizing pneumonia associated with invasive pulmonary aspergillosis: a case report and review of the literature. ( 25674228 )
2014
30
The spectrum of presentations of cryptogenic organizing pneumonia in high resolution computed tomography. ( 25493105 )
2014
31
Fatal case of cryptogenic organizing pneumonia associated with Everolimus. ( 25827702 )
2014
32
Bronchoalveolar lavage fluid and blood natural killer and natural killer T-like cells in cryptogenic organizing pneumonia. ( 24889556 )
2014
33
Cryptogenic organizing pneumonia in Sweet's syndrome: case report and review of the literature. ( 25196175 )
2014
34
Macrolide use leads to clinical and radiological improvement in patients with cryptogenic organizing pneumonia. ( 24460438 )
2014
35
Cryptogenic organizing pneumonia associated with radiation: A report of two cases. ( 24396439 )
2014
36
Association of cryptogenic organizing pneumonia in bilateral anterior uveitis. ( 25520652 )
2014
37
Clinicopathologic features associated with relapse in cryptogenic organizing pneumonia. ( 24342432 )
2013
38
Autopsy findings of fatal cryptogenic organizing pneumonia. ( 23696931 )
2013
39
Cryptogenic organizing pneumonia in Tomm5(-/-) mice. ( 22688586 )
2013
40
Cryptogenic organizing pneumonia: A rare extra-intestinal manifestation of Crohn's disease. ( 24090908 )
2013
41
Pre-transplant risk factors for cryptogenic organizing pneumonia/bronchiolitis obliterans organizing pneumonia after hematopoietic cell transplantation. ( 23933758 )
2013
42
Cryptogenic organizing pneumonia during adjuvant chemotherapy with oxaliplatin, 5-fluorouracil, and leucovorin (FOLFOX) for colon cancer. ( 22842502 )
2013
43
Cryptogenic organizing pneumonia with fatal respiratory failure. ( 22372175 )
2012
44
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
45
Simultaneous diagnosis of cryptogenic organizing pneumonia and HIV in a 45 year old man. ( 23569519 )
2012
46
Cryptogenic organizing pneumonia. ( 23001801 )
2012
47
Chronic exposure to particles caused bronchioloalveolar carcinoma in a patient with cryptogenic organizing pneumonia evaluated by elemental analysis. ( 21675931 )
2012
48
Anti-synthetase syndrome presenting as cryptogenic organizing pneumonia. ( 26029595 )
2012
49
Reversed halo sign in active pulmonary tuberculosis: criteria for differentiation from cryptogenic organizing pneumonia. ( 22109285 )
2011
50
Cryptogenic organizing pneumonia mimicking malignancy in a patient with rheumatoid arthritis. ( 21885507 )
2011

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:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.73 CXCL8 LAMC2 MMP9 MUC1 TIMP1 TNC
2 collagen-containing extracellular matrix GO:0062023 9.5 LAMC2 MMP9 TNC
3 basement membrane GO:0005604 9.33 LAMC2 TIMP1 TNC
4 extracellular matrix GO:0031012 9.26 LAMC2 MMP9 TIMP1 TNC
5 extracellular region GO:0005576 9.17 CD8A CXCL8 LAMC2 MMP9 MUC1 TIMP1

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 9.16 MMP9 TIMP1
2 extracellular matrix organization GO:0030198 9.13 LAMC2 MMP9 TNC
3 cytokine-mediated signaling pathway GO:0019221 8.92 CXCL8 MMP9 MUC1 TIMP1

Sources for Cryptogenic Organizing Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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