Bronchiolitis obliterans
Synonyms: Constrictive bronchiolitis · Obliterative bronchiolitis
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare lung disorder that is mainly associated with chronic allograft dysfunction after lung transplantation and that is characterized by inflammation and fibrosis of bronchiolar walls that reduce the diameter of the bronchioles and result in progressive and irreversible airflow obstruction.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Bronchiolitis obliterans | Constrictive bronchiolitis · Obliterative bronchiolitis |
| fr | Bronchiolite oblitérante | Bronchiolite constrictive · Bronchiolite obstructive |
| de | Bronchiolitis obliterans | Bronchiolitis, konstriktive · Bronchiolitis, obliterative |
| es | Bronquiolitis obliterante | Bronquiolitis constrictiva |
| it | Bronchiolite obliterante | Bronchiolite costrittiva · Bronchiolite obliterativa |
| nl | Bronchiolitis obliterans | Constrictieve bronchiolitis · Obliteratieve bronchiolitis |
| pt | Bronquiolite obliterante | Bronquiolite constritiva · Bronquiolite obliterativa |
| pl | Zarostowe zapalenie oskrzelików z obturacyjną chorobą płuc | BOOP · Zaciskające zapalenie oskrzelików · Zarostowe zapalenie oskrzelików |
| cs | Obliterující bronchiolitida s obstrukční plicní nemocí | BOOP · Konstriktivní bronchiolitida · Obliterující bronchiolitida |
| uk | Облітеруючий бронхіоліт | Констриктивний бронхіоліт · Облітеруючий бронхіоліт |
| zh | 闭塞性细支气管炎伴阻塞性肺疾病 | 闭塞性细支气管炎 |
| tr | Obstrüktif akciğer hastalığı ile seyreden bronşiyolit obliterans | Konstriktif bronşiolit · Obliteratif bronşiolit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J44.8 | NTBT | attributed | validated |
| ICD-11 | CA26.0 | E | specific | validated |
| MONDO | 15265 | E | validated | |
| UMLS | C0006272 | E | validated | |
| GARD | 5961 | E | validated | |
| MedDRA | 10029888 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:506222Rare disorder potentially indicated for lung transplant
rare transplant-related disorders - ORPHA:101944Rare pulmonary disease
rare respiratory diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
16 annotations · validated 2021-08-26| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0006536 | Airway obstruction | |
| HP:0011946 | Bronchiolitis obliterans | |
| HP:0012735 | Cough | |
| HP:0032359 | Decreased forced expiratory flow 25-75% | |
| HP:0002094 | Dyspnea | |
| HP:0012418 | Hypoxemia | |
| HP:0030877 | Reduced FEV1/FVC ratio | |
| HP:0032342 | Reduced forced expiratory volume in one second | Diagnostic criterion |
| HP:0032341 | Reduced forced vital capacity | |
| Frequent (79-30%) | ||
| HP:0045051 | Decreased DLCO | |
| HP:0031246 | Nonproductive cough | |
| HP:0002090 | Pneumonia | |
| HP:0011947 | Respiratory tract infection | |
| Occasional (29-5%) | ||
| HP:0002960 | Autoimmunity | |
| HP:0002110 | Bronchiectasis | |
| Very rare (<4-1%) | ||
| HP:0025179 | Ground-glass opacification on pulmonary HRCT | |
Run the differential on the 9 obligate / very frequent terms
Annotation source: 30038355[PMID]_24806161[PMID]_26335918[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1303
- This record as JSON: /data/d/1303.json