Bronchiolitis obliterans with obstructive pulmonary disease
General Information (adopted from Orphanet):
| Synonyms, Signs: |
BOOP Constrictive bronchiolitis Obliterative bronchiolitis |
| Number of Symptoms | 29 |
| OrphanetNr: | 1303 |
| OMIM Id: |
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| ICD-10: |
J44.8 |
| UMLs: |
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| MeSH: |
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| MedDRA: |
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| Snomed: |
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Prevalence, inheritance and age of onset:
| Prevalence: | No data available. |
| Inheritance: |
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| Age of onset: |
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Disease classification (adopted from Orphanet):
| Parent Diseases: |
Rare pulmonary disease
-Rare respiratory disease |
Comment:
| Constrictive bronchiolitis is a fibrotic disorder involving terminal and respiratory bronchioles. Most cases of constrictive bronchiolitis occur in lung transplant recipients with chronic allograft rejection or hematopoietic stem cell transplant (HSCT) recipients with graft versus host disease (GVHD) (PMID:23001805). Other causes include exposure to toxic fumes, infections, drugs, and connective tissue diseases (PMID:12457011). The fibrotic constrictive bronchiolitis involves the mid-bronchiole region, whereas the disease 'Cryptogenic organizing pneumonia' usually involves the distal bronchioles extending into alveolar spaces (PMID:20948853). |
Symptom Information:
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(HPO:0012378) | Fatigue | 15332401 | IBIS | 50 / 7739 | ||
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(HPO:0001824) | Weight loss | Frequent [IBIS] | 78% (n=9) | 15332401 | IBIS | 42 / 7739 |
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(HPO:0004415) | Pulmonary artery stenosis | 12457011 | IBIS | 25 / 7739 | ||
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(HPO:0001945) | Fever | Frequent [IBIS] | 44% (n=9) | 15332401 | IBIS | 218 / 7739 |
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(HPO:0100598) | Pulmonary edema | 20948853 | IBIS | 17 / 7739 | ||
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(HPO:0002094) | Dyspnea | Very frequent [IBIS] | 100% (n=29) | 19210653 | IBIS | 132 / 7739 |
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(HPO:0002110) | Bronchiectasis | Occasional [IBIS] | 31% (n=29) | 19210653 | IBIS | 73 / 7739 |
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(HPO:0002113) | Pulmonary infiltrates | Occasional [IBIS] | 7% (n=29) | 19210653 | IBIS | 36 / 7739 |
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(HPO:0012418) | Hypoxemia | 12457011 | IBIS | 18 / 7739 | ||
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(HPO:0006510) | Chronic obstructive pulmonary disease | 20948853 | IBIS | 19 / 7739 | ||
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(HPO:0005348) | Inspiratory stridor | Occasional [IBIS] | 24% (n=29) | 19210653 | IBIS | 8 / 7739 |
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(HPO:0002792) | Reduced vital capacity | 12457011 | IBIS | 17 / 7739 | ||
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(HPO:0002795) | Functional respiratory abnormality | 20948853 | IBIS | 13 / 7739 | ||
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(HPO:0002093) | Respiratory insufficiency | 12457011 | IBIS | 410 / 7739 | ||
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(HPO:0002088) | Abnormality of lung morphology | 15332401 | IBIS | 11 / 7739 | ||
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(HPO:0011950) | Bronchiolitis | 19547903 | IBIS | 8 / 7739 | ||
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(HPO:0002781) | Upper airway obstruction | 23001805 | IBIS | 7 / 7739 | ||
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(HPO:0012416) | Hypercapnia | 12457011 | IBIS | 2 / 7739 | ||
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(HPO:0100730) | Bronchogenic cyst | 22855547 | IBIS | 5 / 7739 | ||
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(HPO:0011946) | Bronchiolitis obliterans | 20948853 | IBIS | 1 / 7739 | ||
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(HPO:0002206) | Pulmonary fibrosis | 9845757 | IBIS | 51 / 7739 | ||
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(HPO:0003326) | Myalgia | 15332401 | IBIS | 143 / 7739 | ||
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(MedDRA:10044100) | Total lung capacity decreased | 20948853 | IBIS | 1 / 7739 | ||
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(MedDRA:10047924) | Wheezing | Frequent [IBIS] | 31% (n=29) | 19210653 | IBIS | 8 / 7739 |
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(HPO:0012735) | Cough | Occasional [IBIS] | 17% (n=29) | 19210653 | IBIS | 24 / 7739 |
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(OMIM) | Cough, nonproductive | 12457011 | IBIS | 5 / 7739 | ||
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(MedDRA:10059411) | Prolonged expiration | Frequent [IBIS] | 34% (n=29) | 19210653 | IBIS | 1 / 7739 |
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(MedDRA:10059487) | Lung hyperinflation | Occasional [IBIS] | 7% (n=29) | 19210653 | IBIS | 2 / 7739 |
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(MedDRA:10067182) | Bronchial wall thickening | 15332401 | IBIS | 5 / 7739 |
Associated genes:
ClinVar (via SNiPA)
| Gene symbol | Variation | Clinical significance | Reference |
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