Cryptogenic organizing pneumonia

General Information (adopted from Orphanet):

Synonyms, Signs: COP
BOOP
Bronchiolitis obliterans-organizing pneumonia
Number of Symptoms 45
OrphanetNr: 1302
OMIM Id:
ICD-10: J84.8
UMLs:
MeSH: D018549
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance:
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: Idiopathic interstitial pneumonia
 -Rare respiratory disease

Comment:

Cryptogenic organizing pneumonia (COP) is an interstitial lung disease characterized by intra-alveolar buds of connective tissue (PMID:25674228). Clinical features and radiological images resemble infectious pneumonia, however, there is no response to antibiotics (PMID:25493105). COP characteristically responds to broad-spectrum anti-inflammatory corticosteroids and is therefore considered an inflammatory disease (PMID:24460438). The disease usually involves the distal bronchioles extending into alveolar spaces, whereas the 'fibrotic constrictive bronchiolitis' involves the mid-bronchiole region (PMID:20948853).

Symptom Information: Sort by abundance 

1
 (HPO:0012378) Fatigue 1623812 IBIS 50 / 7739
2
 (HPO:0100749) Chest pain 25560867 IBIS 92 / 7739
3
 (HPO:0001824) Weight loss 25560867 IBIS 42 / 7739
4
 (HPO:0001667) Right ventricular hypertrophy 23696931 IBIS 23 / 7739
5
 (HPO:0001648) Cor pulmonale 23696931 IBIS 16 / 7739
6
 (HPO:0001974) Leukocytosis 23696931 IBIS 33 / 7739
7
 (HPO:0012176) Abnormality of natural killer cells 24889556 IBIS 2 / 7739
8
 (HPO:0003565) Elevated erythrocyte sedimentation rate 10602956 IBIS 31 / 7739
9
 (HPO:0011227) Elevated C-reactive protein level 25674228 IBIS 55 / 7739
10
 (HPO:0001945) Fever Frequent [IBIS] 47% (n=72) 23114922 IBIS 218 / 7739
11
 (HPO:0002789) Tachypnea 8252981 IBIS 48 / 7739
12
 (HPO:0002111) Restrictive deficit on pulmonary function testing Frequent [IBIS] 24424167 IBIS 25 / 7739
13
 (HPO:0011945) Bronchiolitis obliterans organizing pneumonia Very frequent [IBIS] 24342432 IBIS 2 / 7739
14
 (HPO:0006530) Interstitial pulmonary disease Very frequent [IBIS] 24424167 IBIS 26 / 7739
15
 (HPO:0002097) Emphysema Frequent [IBIS] 62% (n=26) 24342432 IBIS 40 / 7739
16
 (HPO:0005952) Decreased pulmonary function Very frequent [IBIS] 24342432 IBIS 8 / 7739
17
 (HPO:0002792) Reduced vital capacity 21702658 IBIS 17 / 7739
18
 (HPO:0002093) Respiratory insufficiency Very frequent [IBIS] 25674228 IBIS 410 / 7739
19
 (HPO:0002206) Pulmonary fibrosis Frequent [IBIS] 81% (n=26) 24342432 IBIS 51 / 7739
20
 (HPO:0002113) Pulmonary infiltrates 25674228 IBIS 36 / 7739
21
 (HPO:0002202) Pleural effusion Occasional [IBIS] 24% (n=72) 23114922 IBIS 22 / 7739
22
 (HPO:0002094) Dyspnea Frequent [IBIS] 57% (n=72) 23114922 IBIS 132 / 7739
23
 (HPO:0002207) Diffuse reticular or finely nodular infiltrations Frequent [IBIS] 42% (n=26) 24342432 IBIS 11 / 7739
24
 (HPO:0012418) Hypoxemia Occasional [IBIS] 27% (n=26) 24342432 IBIS 18 / 7739
25
 (HPO:0012415) Abnormal blood gas level 24460438 IBIS 9 / 7739
26
 (HPO:0002110) Bronchiectasis Occasional [IBIS] 15% (n=26) 24342432 IBIS 73 / 7739
27
 (HPO:0002102) Pleuritis Occasional [IBIS] 19% (n=26) 24342432 IBIS 7 / 7739
28
 (HPO:0002105) Hemoptysis Occasional [IBIS] 15% (n=72) 23114922 IBIS 30 / 7739
29
 (HPO:0002795) Functional respiratory abnormality Frequent [IBIS] 68% (n=94) 3965933 IBIS 13 / 7739
30
 (HPO:0002109) Abnormality of the bronchi 24342432 IBIS 6 / 7739
31
 (HPO:0030166) Night sweats 14533463 IBIS 3 / 7739
32
 (HPO:0012735) Cough Frequent [IBIS] 53% (n=72) 23114922 IBIS 24 / 7739
33
 (OMIM) Pulmonary fibrosis with fibroblast foci on histology 23001801 IBIS 3 / 7739
34
 (MedDRA:10062049) Lymphocytic infiltration 23696931 IBIS 9 / 7739
35
 (MedDRA:10054949) Metaplasia Occasional [IBIS] 19% (n=26) 24342432 IBIS 1 / 7739
36
 (OMIM) Radiograph shows ground glass appearance Frequent [IBIS] 15459629, 25493105 IBIS 7 / 7739
37
 (MedDRA:10047924) Wheezing Occasional [IBIS] 8% (n=72) 23114922 IBIS 8 / 7739
38
 (OMIM) Reduced forced vital capacity 11961300 IBIS 3 / 7739
39
 (OMIM) Reduced diffusion capacity Frequent [IBIS] 86% (n=94) 25674228 IBIS 8 / 7739
40
 (OMIM) Cough, nonproductive 25560867 IBIS 5 / 7739
41
 (MedDRA:10068496) Natural killer cell count increased 24889556 IBIS 1 / 7739
42
 (MedDRA:10025080) Lung consolidation Frequent [IBIS] 57% (n=72) 23114922 IBIS 5 / 7739
43
 (MedDRA:10065906) Carbon monoxide diffusing capacity decreased 8685496 IBIS 8 / 7739
44
 (MedDRA:10016987) Forced expiratory volume decreased 11961300 IBIS 2 / 7739
45
 (MedDRA:10025482) Malaise Occasional [IBIS] 13% (n=72) 23114922 IBIS 4 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: